Practice Essentials

Henoch-Schnlein purpura (HSP) is an acute immunoglobulin A (IgA)mediated disorder

characterized by a generalized vasculitis involving the small vessels of the skin, the
gastrointestinal (GI) tract, the kidneys, the joints, and, rarely, the lungs and the central
nervous system (CNS). See the image below.

Characteristic rash of Henoch-

Schnlein purpura.
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Signs and symptoms

The typical prodrome of HSP includes the following:

Headache
Anorexia
Fever

Subsequently, symptoms develop, of which the following are the most common:

Rash (95-100% of cases), especially involving the legs; this is the hallmark of the
disease
Abdominal pain and vomiting (35-85%)
Joint pain (60-84%), especially involving the knees and ankles
Subcutaneous edema (20-50%)
Scrotal edema (2-35%)
Bloody stools

Because HSP can affect all organ systems, a full physical examination is indicated. Physical
findings in HSP may include the following:

Skin findings (usually the first sign of HSP) - Erythematous macular or urticarial
lesions, progressing to blanching papules and later to palpable purpura; typically
symmetrical and tend to be distributed in dependent body areas, such as the ankles
and lower legs in older children and adults and the back, buttocks, upper extremities,
 and upper thighs in young children; hives, angioedema, and target lesions can also
occur
Renal findings - Acute glomerular lesions, including mesangial hypercellularity,
endocapillary proliferation, necrosis, cellular crescents, and leukocyte infiltration
Gastrointestinal (GI) findings - Abdominal pain, melena, bloody diarrhea,
hematemesis, duodenal ulcers, and massive GI hemorrhage
Joint findings - Arthralgia and swelling
Arthralgia is the presenting feature in as many as 25% of cases. Joints may be
swollen, tender, and painful. Warmth, erythema, and effusions are not typically
associated with HSP. The knees and ankles are most commonly affected. On rare
occasions, symptoms involve the fingers and wrists. Findings are transient but can
occur again during active disease. The joints are not permanently deformed.
Other findings - Vasculitis involving the myocardium or lungs; stenosing ureteritis,
priapism, penile edema, or orchitis; vasculitis involving the central nervous system
(CNS) and intracranial hemorrhage; bilateral subperiosteal orbital hematomas; adrenal
hematomas; acute pancreatitis as the sole presenting feature (rare); cystic changes of
the ovaries

See Presentation for more detail.

Diagnosis

No specific diagnostic laboratory test is available to assess for markers of HSP. The
following general laboratory tests may be helpful for excluding other diagnoses and
evaluating renal function:

Antinuclear antibody (ANA) and rheumatoid factor (RF)

Factors VIII and XIII
Urinalysis
Complete blood count (CBC)
Platelet count
Erythrocyte sedimentation rate (ESR)
Stool guaiac test
Blood urea nitrogen (BUN) and creatinine
Amylase and lipase
Electrolytes
Plasma D-dimer
Plasma thrombin-antithrombin (TAT) complex, prothrombin fragment (PF)-1, and
PF-2
Prothrombin time (PT) and activated partial thromboplastin time (aPTT)
Serum IgA
Antistreptolysin O (ASO)
CH50
C3 and C4
Immunocomplexes of IgG and IgA

Imaging modalities that may be considered include the following:

Ultrasonography (abdominal, scrotal/testicular)

 Radiography (chest radiography; plain radiography of the abdomen; contrast
radiography of the small intestine; barium enema study)
Magnetic resonance imaging (MRI; for assessing neurologic findings)
Computed tomography (CT) of the head or abdomen

Other studies that may be warranted are as follows:

Endoscopy
Renal biopsy (particularly when nephrotic syndrome persists and when renal function
deteriorates)

See Workup for more detail.

Management

Treatment remains primarily supportive in most cases, though pharmacotherapy,

plasmapheresis, and surgical interventions may also be considered in select cases.

Supportive measures may include the following:

Ensuring adequate hydration

Monitoring for abdominal and renal complications
Treating minor symptoms of arthritis, edema, fever, or malaise
Eating a bland diet
Discontinuance of any drugs suspected of playing a causative role

Joint and soft tissue discomfort may be reduced by giving analgesics, such as the following:

Acetaminophen
Ibuprofen
Flurbiprofen
Ketoprofen
Naproxen

Corticosteroids may be considered in the following situations:

Persistent nephrotic syndrome

Crescents in more than 50% of glomeruli
Severe abdominal pain
Substantial GI hemorrhage
Severe soft tissue edema
Severe scrotal edema
Neurologic system involvement
Intrapulmonary hemorrhage

Other treatment regimens have included IV or oral steroids with or without any of the
following:

Azathioprine
Cyclophosphamide
 Cyclosporine
Dipyridamole
High-dose IV immunoglobulin G (IVIg)
Danazol
Fish oil

Plasmapheresis may be effective in delaying the progression of kidney disease.

Surgical interventions that may be considered in specific circumstances include the

following:

Surgery for severe bowel ischemia

Kidney transplantation for severe renal disease that is resistant to medical therapy
Tonsillectomy together with corticosteroid pulse therapy for progressive HSP
nephritis

Background
Henoch-Schnlein purpura (HSP; also referred to as Schnlein-Henoch purpura,
anaphylactoid purpura, or purpura rheumatica) is an acute immunoglobulin A (IgA)
mediated disorder characterized by a generalized vasculitis involving the small
vessels of the skin, the gastrointestinal (GI) tract, the kidneys, the joints, and, rarely,
the lungs and the central nervous system (CNS). [1, 2, 3] It is a subset of necrotizing
vasculitis characterized by fibrinoid destruction of blood vessels and leukocytoclasis.
The prevalence of HSP peaks in children aged 3-10 years, but the condition is also
seen in adults. [4] In the Northern hemisphere, the disease occurs mostly between
November and January. The male-to-female ratio is 1.5-2:1.
The dominant clinical features of HSP include cutaneous purpura, arthritis, abdominal
pain, GI bleeding, orchitis, and nephritis. In one half to two thirds of children, an
upper respiratory tract infection (URTI) precedes the clinical onset of HSP by 1-3
weeks. In general, patients with HSP appear mildly ill. They often have a fever, with a
temperature that usually does not exceed 38C (100.4F).
HSP is typically an acute, self-limited illness, and treatment is primarily supportive.
However, one third of patients have 1 or more recurrences.

Pathophysiology
IgA clearly plays a critical role in the immunopathogenesis of HSP, as evidenced by
increased serum IgA concentrations, IgA-containing circulating immune complexes, and IgA
deposition in vessel walls and renal mesangium. HSP is almost exclusively associated with
abnormalities involving IgA1, rather than IgA2. The predominance of IgA1 in HSP may be a
consequence of abnormal glycosylation of O-linked oligosaccharides unique to the hinge
region of IgA1 molecules.
IgA aggregates or IgA complexes with complement deposited in target organs, resulting in
elaboration of inflammatory mediators, including vascular prostaglandins such as
prostacyclin, may play a central role in the pathogenesis of HSP vasculitis.

A subpopulation of human lymphocytes bears surface Fc and/or C3 receptors (complement

receptor lymphocytes), which can bind circulating immune complexes or C3 generated by
activation of the alternative complement pathway. Such immune complexes appear in HSP
and may be part of the pathogenetic mechanism.

Some have speculated that an antigen stimulates the production of IgA, which, in turn, causes
the vasculitis. Allergens, such as foods, horse serum, insect bites, exposure to cold, and drugs
(eg, ampicillin, erythromycin, penicillin, quinidine, and quinine), may precipitate the illness.

Infectious causes include bacteria (eg, Haemophilus parainfluenzae, Mycoplasma,

Legionella, Yersinia, Shigella, or Salmonella) and viruses (eg, adenoviruses, Epstein-Barr
virus [EBV], parvoviruses, or varicella-zoster virus [VZV]). Vaccines such as those against
cholera, measles, paratyphoid A and B, typhoid, and yellow fever have also been implicated.
Evidence supporting a direct role of herpesvirus, retrovirus, or parvovirus infection in the
pathogenesis of HSP is lacking.

Alterations in the production of interleukins (ILs) and growth factors may also play a
pathogenetic role. Tumor necrosis factor (TNF), IL-1, and IL-6 may mediate the
inflammatory process present in HSP. Transforming growth factor (TGF) is a recognized
stimulant of IgA production. The elevated levels of hepatocyte growth factor present during
the acute phase of HSP may reflect endothelial-cell damage or dysfunction. Increased levels
of vascular endothelial growth factor (VEGF) may at least partly induce these changes.

Cytokines have been implicated in the pathogenesis of HSP, and endothelins (ETs), which
are vasoconstrictor hormones produced by endothelial cells, may also have a role. levels of
ET-1 are substantially higher during the acute phase of the disease than during remission or
in a control group of children. However, ET-1 levels do not appear to be correlated with
morbidity, severity of disease, or acute-phase reactant response.

Although several lines of evidence suggest a genetic susceptibility to HSP, the fundamental
basis for this abnormality remains unclear.

A functional correlation of the IL1RN-2 allele and IL-1ra production in patients with IgA
nephropathy and HSP nephritis (HSPN) has been described. Therefore, gene polymorphism
may contribute to the diversity of clinical responses to inflammatory stimulation.

The prevalence of the human parvovirus B19 component NS1 gene in patients with HSP and
hypersensitivity vasculitis is increased.

Results support a role of human leukocyte antigen (HLA)-B35 in the susceptibility to

nephritis in unselected patients with HSP.

Researchers are currently investigating the importance of nitric oxide (NO) production in
disease activity. Inducible NO synthase polymorphism has been associated with susceptibility
to HSP in northwestern Spain. Aliyazicioglu et al have suggested that leptin and NO may
play a role in the immunoinflammatory process of HSP, especially in the acute phase. [5]
HSP that is likely due to montelukast has been noted in patients who present with subacute
intestinal obstruction.

Yilmaz et al examined 28 children with HSP and 79 healthy children to evaluate activities of
protein C, free-protein S, and antithrombin; resistance to activated protein C; and levels of
fibrinogen. [6] D-dimer, thrombin-antithrombin (TAT) complex, prothrombin fragment (PF)-1,
PF-2, and von Willebrand factor antigen (vWAg) and its activity (RiCof) were also
investigated.

The investigators found that in patients with HSP, fibrinogen, D-dimer, TAT complex, PF-1,
PF-2, vWAg, and RiCof levels were significantly higher during the acute phase than during
the recovery phase and were significantly higher than those of control subjects. [6] The
severity of disease was significantly correlated with TAT, PF-1, PF-2, vWAg, and D-dimer
levels.

Higher levels of matrix metalloproteinase (MMP)-9 levels in urine and serum appear to
increase nephrologic severity in children with HSP.

Use of TNF- blockers such as adalimumab may increase the risk of developing HSP.

HSP versus IgA nephropathy

HSP and IgA nephropathy appear to be related disorders. However, the precise relation
between them requires further definition. The question has been raised as to whether HSP and
IgA nephropathy are 2 aspects of a single disease entity or 2 distinct entities. The following
commonalities and differences have been noted:

IgA nephropathy almost exclusively involves young adults and typically affects only the
kidneys, whereas HSP affects mostly children and involves the skin and connective tissues, GI
tract, joints, and scrotum, as well as the kidneys [3, 7, 8]
The occurrence of extrarenal manifestations in IgA nephropathy is similar to that in HSP
IgA nephropathy has developed in patients with a history of HSP, and HSP and IgA
nephropathy have occurred in the same families; in a survey of 40 families in which 2 or
more members had IgA nephropathy, 5 presented with HSP [9]
Patients with HSP who undergo renal transplantation develop IgA deposits in the graft
The prevalence of both conditions is high in certain geographic areas
Similar changes in the IgA system (ie, high IgA, IgA-1C, IgA1-IC, IgA-fibronectin aggregates,
aberrantly glycosylated IgA in the circulation) occur in the 2 diseases [10, 11, 12]
Cystic changes in the ovaries of a prepubertal girl with HSP have been recorded

Overall, the data tend to support the view that HSP and IgA nephropathy are distinct diseases.
[13]
Zhou et al examined 31 children aged 3-15 years with IgA nephropathy and 120 children
aged 4-15 years with HSP, noting their clinical manifestations, blood biochemistries, serum
immunology, and follow-up data. [14] Renal pathologic findings on light microscopy,
immunofluorescence study, and electron microscopy were analyzed and compared between
31 children with IgA nephropathy and 32 children with HSP.

The age of onset was greater than 12 years in 25.8% of the children with IgA nephropathy but
in only 10% of those with HSP. [14] Clinical patterns of IgA nephropathy were similar to those
of HSP, but extrarenal manifestations were observed more often in patients with HSP.
All of the HSP patients had skin purpura, 59% had GI symptoms, and 47% had arthralgia.
Abdominal pain occurred in only 3.2% of children with IgA nephropathy. [14] In patients with
IgA nephrology and in patients with HSP, renal pathology revealed global sclerosis in 35.5%
and 3.1%, mesangial sclerosis in 41.9% and 6.3%, endothelial proliferation in 29% and
65.6%, and thin basement-membrane nephropathy in 6.5% and 0%, respectively.

In HSP, electronically dense deposits in HSP were sparse, loose, and widely spread in the
glomerular mesangium, in the subendothelial area, and even in the intrabasement membranes,
whereas in IgA nephropathy, the deposits were dense, lumpy, and mostly limited to
mesangium and paramesangium. [14]

Immunoglobulin G (IgG) was found in glomerular immune deposits in 71.9% of patients with
HSP but in only 19.4% of patients with IgA nephropathy. [14] No IgG deposit was observed in
81.6% of those with IgA nephropathy; most had IgA and immunoglobulin M (IgM) or C3
deposits. Predominant IgG deposits were found in 12.5% of HSP patients, with relatively
weak IgA deposits. Moreover, 6.3% of HSP patients had linear IgG deposits in the
glomerular capillary wall, a finding that was not noted in patients with IgA nephropathy.

The rate of complete remission was 72.5% in patients with HSP at an average of 20 months
follow-up; the corresponding rate was 19.4% in those with IgA nephropathy after 34 months
follow-up. [14] Moreover, 64.5% of patients with IgA nephropathy had consistent hematuria
and proteinuria, and 16.1% had active nephritides.

The important clinicopathologic differences Zhou et al found between HSP and IgA
nephropathy argue against the single-disease hypothesis.

Etiology
The etiology of HSP remains to be clearly defined but is thought to be multifactorial, with
genetic, environmental, and antigenic components. More than 75% of patients report
antecedent URTI, pharyngeal infection, or GI infection. Multiple bacterial and viral
infectious agents have been associated with the development of HSP, and cases also have
been reported after drug ingestions and vaccinations. [15]

Infections that may precede the development of HSP include the following:

Mononucleosis
Group A streptococcal infection (most common)
Hepatitis
Mycoplasma infection
Campylobacter enteritis
Hepatitis Crelated liver cirrhosis
Subacute bacterial endocarditis
Helicobacter pylori infection [16, 17, 18] (specifically noted in China [19] )
Yersinia infection
Shigella infection
 Salmonella infection
Brucellosis
Legionella species
Parvovirus
Adenovirus
EBV infection
VZV infection

Vaccinations that may precede the development of HSP include the following:

Typhoid and paratyphoid A and B

Measles
Yellow fever
Cholera

Environmental exposure to the following may precede the development of HSP:

Drugs (eg, ampicillin, erythromycin, penicillin, quinidine, quinine, losartan, and

cytarabine [20] )
Foods
Horse serum
Cold exposure
Insect bites

Glomerulocystic kidney disease has also been noted.

Epidemiology
United States statistics

In the United States, the prevalence of HSP is approximately 14-15 cases per 100,000
population.

International statistics

In the United Kingdom, the estimated annual incidence of HSP is 20.4 cases per 100,000
population. [21] In a Norwegian community hospital, the prevalence of Henoch-Schoenlein
purpura was 3.3 cases per 100,000 inhabitants. [22]

In a study that examined the renal biopsy results of 65 children younger than 18 years
obtained by the Clinical Hospital in the Croatian region of Dalmatia over a 10-year period
(1995-2005), 10.8% of glomerulonephritis cases were due to HSP. [23]

Nong et al reviewed the records of 107 Taiwanese pediatric patients diagnosed with HSP
between 1991 and 2005 who had a mean age of 6.2 2.5 years (range, 2-13 years); the male-
to-female ratio was 1:0.7. [24] The primary symptoms included the following:
 Rashes (95.3%)
GI symptoms (72.0%)
Joint involvement (46.7%)
Kidney involvement (28.0%)

The most common first manifestations were as follows:

Rashes (56.1%)
GI symptoms (35.5%)
Joint involvement (12.1%)

From January 1983 to June 2004, Suehiro et al followed 4502 patients at the Pediatric
Rheumatology clinic in Brazil. [25] A diagnosis of HSP was made in 203 cases (4.5%); 5
patients (0.1%) had acute hemorrhagic edema of infancy (AHEI). All patients with AHEI
were male, and the mean age at onset was 18 months (range, 8-21 months).

Age-related demographics

HSP primarily affects children; it may be seen in adults, but much less frequently. [2, 8] In the
United States, the prevalence peaks in children aged 5 years. Approximately 75% of cases
occur in children aged 2-11 years; HSP is rare in infants and young children. Older age at the
onset of HSP is associated with the development of chronic renal disease. [26] AHEI, a related
but milder condition, occurs in infants younger than 2 years. [27]

Ghrahani et al conducted a retrospective study in children with HSP in Hasan Sadikin

Hospital from 2006 to 2011 to evaluate renal involvement in children with HSP. The authors
reported that there were 128 patients with an age range from 6 month to 15 years. Peak
morbidity was between 5-10 years old. In most patients (71%) purpura was the first
symptom. Seventy-one patients (44.5%) had arthritis and 89 patients (69.5%) had abdominal
pain, while renal involvement was in 28 patients (21.8%). Gastrointestinal manifestations
tend to manifest in patients less than 5 years old, while renal involvement tend to manifest in
age group 11-15 years old. The authors concluded that renal involvement in children with
HSP is more common in age group 11 to 15 years old. [28]

Sex-related demographics

HSP occurs more often in boys than in girls. In children, the male-to-female ratio is 1.5-2:1.
In adults, the male-to-female ratio is approximately 1:1.

Race-related demographics

Whites are affected more often than blacks.

In a study from Thailand, patients most commonly presented between the ages of 3 and 5
years. [29] Frequency peaked from December to February. Organs involved included the skin
(100%), GI tract (74.5%), and kidneys (46.8%). Joints were also affected (42.6%). Renal
involvement was detected within the first 2 months in 16 patients (72.7%); however, it was
delayed until 6 months after diagnosis in 6 patients.
No risk factors for renal involvement could be identified in this study. [29] Mean follow-up
time was 2.6 years (range, 1-5 years). Residual renal disease occurred in 6 (38%) of 16
patients, but none had end-stage renal disease (ESRD).

In a study from China, a male predominance was observed in children but not in adults. [30]
Preceding infection was noted in 40.5% of children and 31.6% of adults; 8.3% of children
and 13.2% of adults were receiving medication at the onset of the disease.

The investigators found that abdominal pain was more common in children than adults
(70.2% vs 28.9%), but renal involvement was more common and severe in adults than in
children; this involvement manifested as frequent hypertension and heavy proteinuria. [30]
During acute attacks, leukocytosis, thrombocytosis, and elevated serum C-reactive protein
(CRP) levels were most frequently observed in children, whereas elevated serum IgA and
cryoglobulin levels were most common in adults.

A study of 450 cases from Turkey showed that girls, patients with atypical presentations, and
patients undergoing early corticosteroid treatment had an increased risk of developing kidney
disease; relapses occurred more often in children treated with corticosteroids. [31]

Familial kindreds with HSP have been noted in Taiwanese aboriginal people. [32]

Prognosis
HSP is generally a benign disease with an excellent prognosis. Spontaneous resolution is
usual: Most patients experience complete resolution of symptoms within 8 weeks, and
probably fewer than 5% experience chronic symptoms. Initial attacks of HSP can last several
months, and relapses are possible. HSP is fatal only in the rarest of cases.

A clinical course with complete resolution of the disease usually occurs in patients with the
following:

Mild kidney involvement

No neurologic complications
Disease that lasts less than 4-6 weeks initially

Children younger than 3 years usually have a shorter, milder course than older patients do, as
well as fewer recurrences.

Recurrences occur in as many as 50% of patients within 6 weeks but can happen as late as 7
years after the initial disease. A study by Calvo-Ro et al indicated that in patients with HSP,
the chance of relapse is greater in those with GI and joint manifestations, with 72.3% of
patients in the study with abdominal pain relapsing, compared with 62.3% of those who did
not, and 27.8% of patients with joint manifestations relapsing, compared with 15.5% of those
who did not. [33]

Although HSP generally resolves without permanent consequences, serious GI and renal
complications may occur, and the higher the number of recurrences, the higher the likelihood
of permanent renal damage. Potential GI complications include intussusception (usually
ileoileal), bowel infarction, bowel perforation, hydrops of the gallbladder, pancreatitis, and
massive GI bleeding.

Kidney damage related to HSP is the primary cause of morbidity and mortality. As many as
15% of patients may have long-term renal insufficiency, but no more than 1-2% will have
ESRD. As many as 20% of children who have HSP and are treated in specialized centers
require hemodialysis. The renal prognosis appears to be worse in adults than in children (in
particular, those aged 6 years).

Predictors of serious nephropathy or ESRD include the following:

Bloody stools
Rash persistence
Hematuria and proteinuria (patients with only hematuria do not develop ESRD, but
about 15% of patients with hematuria and proteinuria do develop ESRD)
Signs of nephritis or nephrotic syndrome (50% of patients progress to ESRD within
10 years)
Renal biopsy with extensive glomerular crescents

Patients with a normal urinalysis at 6 months and without previous renal involvement have
not gone on to develop kidney problems. [34]

Pregnant women who had HSP during childhood appear to be at increased risk for developing
hypertension and proteinuria during pregnancy. [35]

Patient Education
Patients should be informed that the disease is most likely to resolve with few residual
adverse effects but that relapses are possible. The clinician should explain that severe kidney
involvement is rare but that if it does occur, aggressive treatment may be required.

For patient education resources, see Blood in the Urine.

History
Henoch-Schnlein purpura (HSP) typically has a prodrome, which includes the following:

Headache
Anorexia
Fever

After the prodrome, a number of symptoms develop, of which the following are the most
common:
 Rash (95-100% of cases), especially involving the legs
Abdominal pain and vomiting (35-85%)
Joint pain (60-84%), especially involving the knees and ankles
Subcutaneous edema (20-50%)
Scrotal edema (2-35%)
Bloody stools

The hallmark of the disease is the characteristic rash (see the image below), which appears in
nearly all patients (though in as many as 50% of children, it may not be the presenting
feature). The rash typically appears in crops, with new crops appearing in waves. Eruptions
usually last an average of 3 weeks.

Characteristic rash of Henoch-

Schnlein purpura.

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Lesions tend to occur on the buttocks and upper thighs in younger children and on the feet,
ankles, and lower legs in older children and adults. The primary differences between children
and adults appear to be the chronicity and severity of the eruption in the latter population;
bullae and ulcers are more common in adults, and cutaneous exacerbations may be seen for 6
months or longer. [36] Chan et al noted a case of HSP presenting as painful bullae on both legs.
[37]

Gastrointestinal (GI) symptoms may accompany the onset of HSP or may develop later in the
course of disease. The most common such symptom is colicky abdominal pain. GI problems
usually follow the onset of rash and joint pain. Multiple and recurrent intestinal perforations
are an unusual complication of HSP. In addition to abdominal pain, GI findings may include
the following:

Nausea
Vomiting
Diarrhea with gross or occult blood
Hematemesis
Intussusception - This occurs in 2-3% of patients, and the lead point can be a submucosal
hematoma
Bowel infarction, with or without perforation
Ileal stricture
 Ileus with massive GI hemorrhage
Acute appendicitis [38, 39]

Arthralgias occur in 60-84% of patients with HSP and are the presenting complaint in
approximately 25% of children. The large joints (eg, the knees and ankles) are the ones most
commonly involved, with pain and edema being the only symptoms; the wrists and fingers
are less commonly involved. True arthritis does not occur, and joint effusions are rare.
Generally, the arthritis resolves completely over several days without permanent articular
damage.

Acute hemorrhagic edema of infancy (AHEI) usually occurs in infants aged 4-24 months,
often after drug ingestion or infection. Onset is dramatic, with acute palpable purpura,
ecchymoses, and tender edema of the limbs and face. Fever, if present, remains mild. Infants
remain hemodynamically stable. Dermatologic findings are notable for a cockadelike rosette-
shaped pattern of macular-papular-hemorrhagic lesions on the face, auricles, and extremities,
which usually appear in successive crops and display varying stages of evolution at any given
time.

Subcutaneous edema is most common in infants. Urticaria, petechiae, and ear lobe necrosis
are additional rare skin manifestations of AHEI. Visceral involvement is rare.

Scrotal involvement is not uncommon in HSP and may mimic testicular torsion, which must
be excluded. Male patients may have associated inflammation and hemorrhage of the testes,
appendix testes, spermatic cord, epididymis, or scrotal wall. True torsion is rare. Ha and Lee
reported that neurologic symptoms, localized edema, and high serum C3 levels were
significantly related to scrotal involvement in male patients with HSP. [40]

In women with HSP, gynecologic symptoms can include painful menstruation.

HSP can be accompanied by neurologic manifestations, particularly headaches. Ozkaya et al

reported cerebral vasculitis in a child with HSP and familial Mediterranean fever. [41] In rare
cases, HSP can be associated with seizures, paresis, or coma.

Other neurologic manifestations of HSP include altered mental status, apathy, hyperactivity,
irritability, mood lability, somnolence, seizures (partial, complex partial, generalized, or
status epilepticus), and focal deficits (eg, aphasia, ataxia, chorea, cortical blindness,
hemiparesis, paraparesis, or quadriparesis). Polyradiculoneuropathies (eg, brachial plexus
neuropathy or Guillain-Barr syndrome) and mononeuropathies (eg, of the facial nerve,
femoral nerve, peroneal nerve, sciatic nerve, or ulnar nerve) may also occur.

The liver and gallbladder can be involved in HSP. Hepatomegaly, hydrops of the gallbladder,
and cholecystitis may ensue. These may contribute to a patients abdominal pain. Acute
appendicitis has been noted in patients with HSP.

Renal pathology is the most important cause of morbidity and mortality in patients with HSP.
Renal involvement may precede skin manifestations (1-4% of patients) but is usually evident
during the acute phase of the disease, sometimes developing as long as 3 months after the
initial presentation. [42] It may persist for as long as 6 months after the onset of the rash. In
most cases, the severity of nephritis is not related to the extent of other HSP manifestations.
Hemoptysis and hemarthroses can develop in patients who have bleeding abnormalities such
as coagulopathy, factor VIII deficiency, vitamin K deficiency, or hypoprothrombinemia.
They also probably include factor V Leiden, protein C deficiency, and protein S deficiency,
but this has not been documented.

Recurrence of previous HSP

Disease recurrence occurs throughout weeks to months in adults and children. In a large
pediatric study by Allen et al, children older than 2 years had a recurrence rate of 50%,
whereas those younger than 2 years had a recurrence rate of less than 25%. [43]

Prais et al studied 267 children (56.7% males) who were hospitalized secondary to HSP, of
whom 7 (2.7%) had HSP that resulted in hospitalization at least twice. [44] No specific risk
factor for recurrence was determined. The mean age for the first recurrence in that subgroup
was 3.67 years (range, 10 months to 7.4 years), and that for the second was 5.03 years (range,
2.2-10 years), with a mean lag time of 13.5 2.8 months (range, 2-26 months). The duration
of the recurrence was 9-30 days. Resolution took more than 2 weeks in 72% of patients.

Physical Examination
Because HSP can affect all organ systems, a full physical examination is indicated. Purpura
of the skin is the most prominent physical finding in HSP, but renal, GI, and joint
manifestations are commonly present. Other manifestations have also been reported.

Skin findings

In most patients, skin lesions are the first sign of HSP. The eruption commonly begins as
erythematous macular or urticarial lesions, progressing to blanching papules and later to
palpable purpura, usually 2-10 mm in diameter (see the image below). Various stages of
eruption may be present simultaneously. Lesions usually occur in crops and may fade over
several days.

Purpuric papules and plaques of the

lower extremity characteristic of Henoch-Schnlein purpura.

View Media Gallery

Lesions typically are symmetrical and tend to be distributed in dependent body areas, such as
the ankles and lower legs in older children and adults (see the images below), and the back,
buttocks, upper extremities, and upper thighs in young children (because these regions tend to
be dependent in young children). The face, palms, soles, and mucous membranes usually are
spared, except in infants, in whom facial involvement may not be uncommon.

Typical rash distribution of Henoch-Schnlein purpura.

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A 9-year-old boy with Henoch-

Schnlein purpura. Note confluence of purpura around the ankles. Image courtesy of Pamela L Dyne,
MD.

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 A 7-year-old girl with Henoch-
Schnlein purpura. Image courtesy of Pamela L Dyne, MD.

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Hemorrhagic macules, papules, and

patches on the ankle and foot of a child with Henoch-Schnlein purpura.

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Palpable purpura can also be present on the forearms and pinnae. Scalp edema can occur.
Hemorrhagic vesicles and bullae are rare.

Within 12-24 hours, the macules evolve into purpuric lesions that are dusky red and have a
diameter of 0.5-2 cm. The lesions may coalesce into larger plaques that resemble ecchymoses
(see the image below). Color in the areas of purpura progresses from red to purple and then
becomes rust-colored or brown before fading. Recurrences tend to take place in the same
sites as previous lesions.
 Older lesions of Henoch-Schnlein purpura demonstrating
increased extravasation with ecchymoses on dorsal foot and ankle.

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Hives, angioedema, and target lesions can also occur. Vesicular eruptions and swelling and
tenderness of an entire limb have been noted. Erythema multiformelike lesions can be
present. HSP with hemorrhagic bullae in children has been noted.

In children younger than 2 years, the clinical picture may be dominated by edema of the
scalp, periorbital area, hands, and feet (AHEI). The severity of edema is correlated with the
severity of the vasculitis and not with the degree of proteinuria. However, the edema has been
attributed to the enteric loss of protein. The cockades characteristic of AHEI display
variable stages of evolution at any given time and look different from the normal purpura of
HSP.

The subcutaneous edema of AHEI is more common in infants. Urticaria, petechiae, and
necrosis of the ear lobe are additional rare skin manifestations of AHEI. AHEI is rarely
associated with visceral involvement.

Renal findings

The most serious complication of HSP is renal involvement, which occurs in 50% of older
children but is serious in only approximately 10% of patients. In 80% of patients, renal
involvement becomes apparent within the first 4 weeks of illness. Overall, 2-5% of patients
progress to end-stage renal failure (ESRD).

In one series, acute glomerular lesions, including mesangial hypercellularity, endocapillary

proliferation, necrosis, cellular crescents, and leukocyte infiltration, were observed in 41%,
12%, 50%, 29%, and 32% of patients, respectively. [45] Only glomerular necrotizing lesions
and cellular crescents correlated with the renal survival rate and were associated with
clinically significant proteinuria and development of hypertension.

In a prospective study of 223 children with HSP, Jauhola et al reported that 46% developed
renal manifestations. [46] The authors recommended that children with renal involvement be
followed for more than 6 months. In addition, they noted that treatment with prednisone did
not affect the renal manifestations.

Gastrointestinal findings

Abdominal pain and bloody diarrhea may precede the typical purpuric rash of HSP,
complicating the initial diagnosis and even resulting in unnecessary laparotomy. GI
manifestations occur in about 50% of cases and usually consist of colicky abdominal pain,
melena, or bloody diarrhea. Hematemesis occurs less frequently. Intussusception should be
suspected in HSP patients with abdominal pain or melena. Barium enema is frequently
therapeutic.

The duodenum and small intestine are the most frequently involved segments of the GI tract.
Duodenal ulcers also occur. Massive GI bleeding has been reported in HSP. [47] Ileal vasculitis
has also been reported.

Joint findings

Arthralgia is the presenting feature in as many as 25% of cases. Joints may be swollen,
tender, and painful. Warmth, erythema, and effusions are not typically associated with HSP.
The knees and ankles are most commonly affected. On rare occasions, symptoms involve the
fingers and wrists. Findings are transient but can occur again during active disease. The joints
are not permanently deformed.

Other findings

Vasculitis involving the myocardium may occur. Vasculitis may also involve the lungs,
resulting in pulmonary hemorrhage or severe bilateral pulmonary hemorrhage, and it may
cause stenosing ureteritis, priapism, penile edema, or orchitis. Ha and Lee reported that
neurologic symptoms, localized edema, and high serum C3 levels were significantly
correlated with scrotal involvement in male patients with HSP. [40] Vasculitis involving the
CNS and intracranial hemorrhage have been reported.

Bilateral subperiosteal orbital hematomas have been noted. Adrenal hematomas have
occurred. In rare patients, acute pancreatitis is the sole presenting feature of HSP. [48] A case
involving cystic changes of the ovaries and HSP has been reported. [49]

Complications
HSP can involve nearly every organ system. Reported complications include the following:

Myocardial infarction
Pulmonary hemorrhage
Pleural effusion
 Intussusception
GI bleeding
Bowel infarction
Renal failure
Hematuria
Proteinuria
Seizures
CNS bleeding
Mononeuropathies
Recurrence of symptoms, specifically those of renal impairment (rare)

Hematuria, usually microscopic, can be accompanied by mild-to-moderate proteinuria (< 2

g/day). Oliguria, hypertension, and azotemia are rarely present. Nephrotic syndrome (urinary
protein excretion >40 mg/m2/hr) can also occur. In most cases, histologic examination of the
kidneys reveals mesangial proliferation that can be diffuse or focal and segmental. Resolution
of the renal involvement is the focus in these patients.

A study reported that even mild forms of HSP nephritis risk significant long-term proteinuria.
The study also added that very early introduction of angiotensin-converting enzyme
inhibitors/angiotensin receptor blockers may improve the long-term outcome independent of
histological lesions. [50]

GI complications include hydrops of the gallbladder, pancreatitis, and GI bleeding. Surgical

complications include intussusception, bowel infarction, and perforation.

Overall, 5% of patients develop ESRD. Urinary complications include bladder-wall

hematoma, calcified ureter, hydronephrosis, and urethritis.

In a retrospective Korean study of 212 children with HSP, Lee et al found palpable purpura
spots 98.1% of subjects, GI symptoms in 75.0%, joint symptoms in 69.8%, renal involvement
in 26.9%, and nephrotic syndrome in 4.7%. The study also found that patients with severe
gastrointestinal symptoms and children over age 7 years had a significantly greater incidence
of renal involvement and nephrotic syndrome. [51]

\Diagnostic Considerations
In addition to the conditions listed in the differential diagnosis, other problems to be
considered include the following:

Acute abdomen
Bowel infarction or perforation
Drug reactions
Elevated renal function test results (blood urea nitrogen [BUN], creatinine)
Essential mixed cryoglobulinemia
Global organ involvement
Hypersensitivity vasculitis
Leukocytoclastic vasculitis
Primary antiphospholipid syndrome
 Rickettsial diseases
Waldenstrm macroglobulinemia
Wegener granulomatosis

Differentials

Acute Glomerulonephritis

Acute Renal Failure

Bacterial Endocarditis

Child Abuse & Neglect: Physical Abuse

Disseminated Intravascular Coagulation

Idiopathic Thrombocytopenic Purpura

IgA Nephropathy

Inflammatory Bowel Disease

Meningitis

Meningococcal Infections

Mononucleosis

Orchitis

Pancreatitis

Pediatrics, Chicken Pox or Varicella

Pediatrics, Gastroenteritis

Pediatrics, Gastrointestinal Bleeding

Pediatrics, Hand-Foot-and-Mouth Disease

Pediatrics, Intussusception

Pediatrics, Kawasaki Disease

Pediatrics, Meningitis and Encephalitis

Rheumatic Fever

Rheumatoid Arthritis
Rocky Mountain Spotted Fever

Septic Shock

Systemic Lupus Erythematosus

Testicular Torsion

Thrombocytopenic Purpura

Approach Considerations
Diagnosis of Henoch-Schnlein purpura (HSP) is clinical and is not based on laboratory
evaluation. Routine laboratory test results are usually within reference ranges. Some
laboratory studies help in excluding other diagnoses and in evaluating renal function,
including urinalysis, complete blood count (CBC) with platelet count and differential, blood
urea nitrogen (BUN) level, creatinine level, prothrombin time (PT), activated partial
thromboplastin time (aPTT), and lipase level.

Some believe that imaging studies are indicated only if the diagnosis is uncertain. Renal
biopsy may be helpful in selected cases.

Laboratory Studies
No specific diagnostic laboratory test is available to assess for markers of HSP. General
laboratory tests may reveal the following:

Antinuclear antibody (ANA) and rheumatoid factor (RF) - Absent

Factor XIII - Reduced in about 50% of patients
Urinalysis - Hematuria; proteinuria may also be found
CBC - Leukocytosis with eosinophilia and a left shift; thrombocytosis is present in
67% of patients
Platelet count - May be elevated; low platelet levels suggest thrombocytopenic
purpura
Erythrocyte sedimentation rate (ESR) - Variably elevated; may be mildly elevated in
as many as 75% of patients
Stool guaiac test - May reveal occult blood
BUN and creatinine - May be elevated, indicating decreased renal function
Amylase and lipase - May be elevated in patients with pancreatitis
Electrolytes - Generally in the reference range, but may be affected by excessive
vomiting
Plasma D-dimer - May be substantially increased
Plasma thrombin-antithrombin (TAT) complex, prothrombin fragment (PF)1, and
PF-2 - May be abnormal
PT and aPTT - May be reduced (eg, hypoprothrombinemia)
 Serum immunoglobulin A (IgA) - Increased in about 50% of patients during the acute
phase of illness; circulating IgA immune complexes may be present in some patients,
though data supporting the presence of classic antigen-antibody complexes have been
questioned
Factor VIII - Decreased in some patients
Antistreptolysin O (ASO) - Elevated in 30% of patients
CH50 - Decreased in 30% of patients
C3 and C4 - Occasionally decreased
Immunocomplexes of IgG and IgA - May be increased

If serial urine samples are obtained in patients with HSP, microscopic hematuria is usually
found; it is probably present in 100% of the patients. However, frank nephritis appears in
only 20-30% of unselected children. The entire clinical spectrum of glomerulonephritis may
occur in HSP. The most common renal manifestations are hematuria with mild-to-moderate
proteinuria.

Coppo et al reported that when severe proteinuria, hypertension, or crescents are present at
onset, the risk of HSP progression is greater in adults and females and appears linked with
increasing mean proteinuria levels during follow-up, more so than in patients with decreased
renal function at onset. [52] Factor XIII activity seems to correlate with severity of abdominal
manifestations [53] ; thus, measuring such activity helps identify patients with severe
gastrointestinal (GI) manifestations who might benefit from substitution therapy. [54, 55]

Ultrasonography
Abdominal ultrasonography may be used if GI symptoms are present. It may be better than
barium enema for diagnosing intussusception, in that HSP-related intussusception is more
likely to be ileoileal than ileocolic (as is typical of idiopathic intussusception). Diagnosis of
HSP using ultrasonography and radionuclide scanning in a child presenting with bilateral
acute scrotum as the main symptom has been reported. In children with GI involvement of
HSP, dedifferentiated wall thickening on ultrasonography reveals a poor clinical prognosis.
[56]

Imaging of the scrotum by means of ultrasonography or a technetium radionuclide scanning

may be necessary if scrotal edema is a presenting feature. Testicular ultrasonography may
help in assessing the testes for hemorrhage or torsion. Doppler or radionuclide testicular scan
results show normal or increased blood flow in HSP, in contrast to the decreased blood flow
seen in testicular torsion.

Bowel ultrasonographic findings include thickening of the bowel wall, free fluid, and
intussusception. Hydrops of the gallbladder is rarely seen. Renal involvement may result in
no ultrasonographic features. If nephritis or nephrotic syndrome occurs, renal enlargement
with loss of corticomedullary differentiation due to edema is observed.

Radiography
If hemoptysis has been noted, a chest radiograph should be obtained. Chest radiography may
help in determining the presence and extent of pulmonary hemorrhage.
Plain radiography of the abdomen may help in diagnosing intestinal obstruction. Contrast-
enhanced radiography of the small intestine may demonstrate thickened mucosal folds or
small barium flecks. Radiologic GI findings mainly include bowel ischemia with
thumbprinting and bowel-wall edema, which is sometimes visible on abdominal radiographs.

A barium enema study may be warranted for evaluating epigastric pain, hematemesis, and
melena and for confirming and treating intussusception. If barium studies are performed,
typical findings of ischemic colitis may be found, including a narrow colon with thickened
mucosa and thumbprinting.

MRI and CT
Some suggest that magnetic resonance imaging (MRI) is useful for assessing neurologic
findings in HSP. In one case, a 6-year-old boy with HSP presented with depression,
generalized convulsions, and cortical blindness. Sequential MRI revealed bilateral cerebral
ischemic lesions in the cortex and white matter of parieto-occipital lobes caused by vasculitis.

Computed tomography (CT) of the head is necessary if neurologic symptoms or severe

headache persist. CT may also aid in the exclusion of other causes of abdominal pain.

Endoscopy
Endoscopy may be needed to evaluate epigastric pain, hematemesis, and melena. Endoscopy
may reveal multiple irregular ulcers, mucosal redness, and petechiae in the duodenum. The
second part of the duodenum is sometimes predominantly affected. Ulcerating lesions
accompanied by hematomalike protrusions can be detected in patients with
histopathologically proven leukocytoclastic vasculitis.

Renal Biopsy
In some cases, renal biopsy may be useful. It should be performed when nephrotic syndrome
persists (though other manifestations may have subsided) and when renal function
deteriorates. When patients present with nephrotic syndrome, hypertension, and rapidly
deteriorating renal function, biopsy often shows circumferential crescents in most of the
glomeruli. The extent of the crescents is of prognostic importance. Approximately 60% of
patients develop renal failure, either in the acute phase of the disease or after several years.

Histologic Findings
HSP is a vasculitis that often involves the kidneys. Histopathologic features of the skin
lesions in infantile HSP can range from a typical leukocytoclastic vasculitis with or without
fibrinoid necrosis to the less specific findings of a lymphohistiocytic perivascular infiltrate
with extravasation of erythrocytes.

Direct immunofluorescence (DIF) testing is a useful adjunct to histopathology; the yield of a

positive test result is substantially higher when the test is performed within 48 hours of
presentation. Immunofluorescence studies reveal perivascular IgA deposition in almost all
patients; this finding is rare in infantile HSP, in which C3 and IgM are most commonly found
in the affected vessel walls.

Renal histology in HSP varies considerably. In some cases, most glomeruli appear unaffected
on light microscopy; only a few show mesangial proliferation. In instances of moderate renal
involvement, focal and segmental intracapillary and extracapillary proliferation may be
present with adhesions and small crescents. Severe cases are characterized by a diffuse
proliferation with infiltration of neutrophils and circumferential crescents in most of the
glomeruli. Tubular atrophy and interstitial infiltration with mononuclear cells may also be
present.

In most patients, IgA deposits in the mesangium and the walls of cutaneous capillaries are
detected. The IgA deposited in the mesangium is mainly of the IgA1 subclass, though IgA2
deposits are noted in rare cases. In addition to IgA, the deposits in mesangium and cutaneous
capillaries frequently contain C3, IgG, and fibrin. C3 deposits are often accompanied by
properdin, whereas C1Q and C4 are usually not present. This observation suggests that the
complement components have been activated by means of the alternative pathway.

The specific renal pathology of HSP includes the following:

Diffuse hypercellularity
Focal and segmental proliferation
Mesangial proliferation
Minimal change to severe crescentic glomerulonephritis
Segmental sclerosis fibrosis
Mononuclear cell infiltration
Mesangial, subendothelial, and subepithelial deposits
Diffuse glomerular deposits of IgA, C3, fibrin, immunoglobulin G (IgG), properdin,
and immunoglobulin M (IgM)
IgA deposits in the mesangium

Approach Considerations
To date, no form of therapy has been found to shorten the duration of Henoch-Schnlein
purpura (HSP) to any significant degree. Therefore, treatment remains primarily supportive in
most cases. This is consonant with the understanding that HSP is a self-limited disease. The
majority of patients recover quickly (ie, within several weeks) without treatment. [57]

Management of HSP includes adequate hydration; immediate discontinuance of any exposure

to antigenic stimulants (eg, drugs); and follow-up each week for the first month, every other
week for the second month, and monthly thereafter until abnormal urinary findings subside.

Patients with HSP are often admitted to the hospital and monitored for abdominal and renal
complications, which may be severe and may occur precipitously (eg, acute abdomen, acute
scrotum, and renal failure). Hospitalization should be strongly considered for HSP patients
with severe abdominal pain, significant gastrointestinal (GI) bleeding, or marked renal
insufficiency.
HSP may mimic an abdominal emergency and, in its severest form, result in small-bowel
infarction, perforation, or both.

In adults with HSP, permanent renal involvement is not uncommon. Hematuria at disease
onset and persistence of renal manifestations during the occurrence of HSP can be significant
predictors of possible development of renal sequelae. These manifestations, along with other
features (eg, onset in summer, anemia at disease onset, or relapses of the disease), may
predict the development of renal sequelae in most patients. All pregnant women with even
mild renal symptoms at the onset of HSP should be carefully observed during and after
pregnancy.

Nephropathy is treated supportively. Patients fluid and electrolyte balance should be

monitored, their salt intake should be restricted, and antihypertensives should be prescribed
when needed. Various drugs (eg, corticosteroids, azathioprine, and cyclophosphamide) and
plasmapheresis have been used to prevent renal disease from progressing. The results have
been inconsistent. No data from controlled studies are available.

Dietary restrictions have no clear role in the management of HSP. Activities can be
performed as tolerated.

Supportive Management
Treatment of HSP is primarily supportive and includes ensuring adequate hydration and
monitoring for abdominal and renal complications. For minor complaints of arthritis, edema,
fever or malaise, symptomatic treatment is advised, including use of acetaminophen,
elevation of swollen extremities, eating a bland diet, and adequate hydration. All unnecessary
drugs should be discontinued if a drug-related etiology is suspected.

Most patients with self-limited cases can be safely discharged home with close follow-up by
the primary physician. The decision for or against hospital admission depends on the
physicians customary practice and individual preference. Admission to the hospital is
recommended for control of abdominal pain or vomiting, monitoring of renal function,
confirming a doubted diagnosis, and observation and monitoring. Patients with renal
involvement require close attention to their fluid balance, electrolyte status, and use of
antihypertensives (if indicated).

A study examining prevention and treatment of renal disease in patients with HSP revealed
no significant difference in the risk of persistent kidney disease at 6 months and 12 months in
children given prednisone for 14-28 days upon presentation in comparison with placebo or
supportive treatment. [58] Also, no significant difference was noted in the risk of persistent
renal disease in children given cyclophosphamide compared with supportive treatment and
with cyclosporin compared with methylprednisolone.

Pharmacologic Therapy
Analgesics

Pain control is essential for quality patient care. Analgesia with nonsteroidal anti-
inflammatory drugs (NSAIDs) or acetaminophen may reduce joint and soft tissue discomfort.
Such agents are often effective and do not seem to worsen the purpura; however, they should
be used cautiously in patients with renal insufficiency.

Corticosteroids

Clinicians often use corticosteroids to treat subcutaneous edema and nephritis in HSP, as well
as to ameliorate associated arthralgias and symptoms associated with GI dysfunction.
However, good, large, prospective studies regarding the treatment of HSP are lacking, [58, 59,
60]
and the evidence does not yet support the use of steroids to prevent or treat renal disease.
[61, 62]
Some authors recommend steroids; others do not. Nevertheless, corticosteroids may be
considered in the following situations:

Persistent nephrotic syndrome

Crescents in more than 50% of glomeruli
Severe abdominal pain
Substantial GI hemorrhage
Severe soft tissue edema
Severe scrotal edema
Neurologic system involvement
Intrapulmonary hemorrhage

Prednisone in a dosage of 1 mg/kg/day for 2 weeks and then tapered over 2 more weeks may
shorten the duration of abdominal pain and joint symptoms, but this benefit must be weighed
against the potential adverse effects of steroids. [63, 64]

A review of randomized clinical trials for any intervention used to improve renal disease in
children with HSP noted that data were very limited except for short-term prednisone;
moreover, prednisone had no benefit in preventing serious long-term renal disease. [62]

The long-term prognosis of HSP directly depends on the severity of renal involvement.
Patients with HSP-related renal dysfunction may benefit from therapy. However, prophylaxis
of renal complications in HSP, though interesting, is not currently recommended. Treatment
of overt HSP includes methylprednisolone pulse therapy and prednisone and other
immunosuppressive medications. If prednisone is used, a regimen consisting of 1-2
mg/kg/day PO for 7 days is recommended. Antihypertensives may be indicated with renal
involvement.

Faedda reported favorable results from the following protocols in patients with severe HSP
[65]
:

Induction with 250-750 mg of intravenous (IV) methylprednisolone daily for 3-7 days plus
cyclophosphamide 100-200 mg/d administered orally (PO)
Maintenance with prednisone 100-200 mg PO every other day plus cyclophosphamide 100-
200 mg/d PO 30-75 days
Tapering of prednisone by approximately 25 mg/month (with the cyclophosphamide dose
remaining constant)
Discontinuance of treatment after at least 6 months by abruptly discontinuing
cyclophosphamide and tapering prednisone completely
Other agents

Other treatment regimens have included IV or oral steroids with or without any of the
following:

Azathioprine
Cyclophosphamide
Cyclosporine
Dipyridamole
High-dose IV immunoglobulin G (IVIg)
Danazol
Fish oil

Of these, only cyclophosphamide has been shown to be effective in a randomized controlled

trial. Although some studies have reported success, cyclosporine does not have sufficient
clinical data to establish its utility in this setting. [66]

Azathioprine, mycophenolate mofetil, and urokinase must be tested before their use is
consistently advocated. Guidelines for prescribing azathioprine in dermatology have been
established. [67] No convincing studies have yet been conducted regarding the use of IVIg,
factor XIII administration, antioxidant vitamin E, and fish oil to treat HSP. [68]

A randomized clinical trial of cyclosporine with methylprednisolone pulses in HSP with

nephritis found that cyclosporine was superior and had many fewer complications. [69] A study
of 12 patients with severe HSP nephritis indicated that patients did well with
methylprednisolone at 30 mg/kg/day for 3 days followed by oral corticosteroids at 2
mg/kg/day for 2 months, cyclophosphamide at 2 mg/kg/day for 2 months, and dipyridamole
at 5 mg/kg/day for 6 months. [70]

Some have noted that parvovirus B19associated HSP must be recognized in adults because
the treatment of choice is IV gamma globulin combined with antitumor necrosis factor
(TNF)- therapy. In contrast, immunosuppressive therapy may lead to a persistent or
worsening disease course in these patients.

Massive GI hemorrhage in isolated intestinal HSP that is responsive to IVIg infusion has
been reported. [71] IVIg was used in a complex case of HSP with brain hemorrhage, but more
work must be done to validate the use of this treatment. [72]

Dapsone has been used to treat associated purpuras and arthralgias. Iqbal and Evans found it
to be effective for HSP. [73]

Rituximab has been noted to be a successful treatment for severe refractory chronic HSP. [74]

Factor VIII concentrate has been used to relieve abdominal pain when corticosteroids are
contraindicated. Recombinant factor VIIa has been used with very pronounced factor XIII
deficiency and compartment syndrome.

Treatment of complicated HSP with mycophenolate mofetil has been reported in a series of
patients. [75]
Plasmapheresis
Plasmapheresis may be effective in delaying the progression of kidney disease. A case series
demonstrated good outcomes in adults with severe HSP who were treated with plasma
exchange in addition to steroids. [76] In an uncontrolled study, Shenoy et al reported that
children with severe HSP and IgA nephropathy recover well if treated with plasmapheresis
alone, without the need for immunosuppressive therapy. [77] Plasmapheresis has been useful in
treating rapidly progressive HSP nephritis. [78]

Surgical Intervention
Surgery may be undertaken to treat severe bowel ischemia. Kidney transplantation may be
indicated in patients with severe renal disease that is resistant to medical therapy. Successful
treatment of progressive HSP nephritis with tonsillectomy and corticosteroid pulse therapy
has been reported.

Consultations
Because HSP is a multisystem disease, consultations with the following specialists can be
helpful in diagnosis and treatment:

Dermatologist
Gastroenterologist
Nephrologist (particularly for assistance in determining if dialysis is indicated)
Rheumatologist
Dermatopathologist [79] (the utility of the consultation is limited to untypical or incomplete
manifestations of HSP to buttress positive vascular IgA immunohistochemical evidence of
the deposits)

Consultation is recommended for patients with renal involvement before discharge from the
emergency department and for all patients who appear acutely ill.

Long-Term Monitoring
In all patients, urinalysis and blood pressure monitoring to evaluate for renal involvement
should be continued for up to 6 months after presentation, even if initial urinalysis results
were normal. Once the initial course of prednisone is administered, additional prednisone
appears to have no role.

When terminal renal failure develops, long-term hemodialysis should be instituted until a
kidney is available for transplantation. Mesangial deposits of IgA are common in the graft,
but they rarely lead to clinical manifestations of recurrent glomerulonephritis.

Children who have demonstrated renal manifestations in the acute phase and continue to have
hematuria or proteinuria should be examined every 3-6 months because renal failure or
hypertension can develop up to 10 years after disease onset.
NSAIDs may be administered to address joint problems. Because of the risk of Reye
syndrome, the use of NSAIDs should be discussed with the patients physician.

Medication Summary
Very limited data are available on pharmacologic treatment of Henoch-Schnlein purpura
(HSP). Nonsteroidal anti-inflammatory drugs (NSAIDs) may help joint pain and do not seem
to worsen the purpura; however, they should be used cautiously in patients with renal
insufficiency. Clinicians often use corticosteroids to treat subcutaneous edema and nephritis,
but few prospective placebo-controlled studies have demonstrated their effectiveness. Other
drugs are currently under investigation.

Immunosuppressants
Class Summary

Immunosuppressive agents decrease inflammation by suppressing migration of

polymorphonuclear leukocytes (PMNs) and reversing increased capillary permeability.

Cyclosporine (Gengraf, Neoral, Sandimmune)

View full drug information

Cyclosporine is a cyclic polypeptide immunosuppressant agent consisting of 11 amino acids.

It is produced as a metabolite by the fungus species Beauveria nivea. Chemically,
cyclosporine is designated as [R-[R*,R*-(E)]]-cyclic(L-alanyl-D-alanyl-N-methyl-L-leucyl-
N-methyl-L-leucyl-N-methyl-L-valyl-3-hydroxy-N,4-dimethyl-L-2-amino-6-octenoyl-L--
amino-butyryl-Nmethylglycyl-N-methyl-L-leucyl-L-valyl-N-methyl-L-leucyl).

Methylprednisolone (Solu-Medrol, Medrol, A-Methapred, Depo-Medrol)

View full drug information

Methylprednisolone decreases inflammation by suppressing PMN migration and reversing

increased capillary permeability.

Prednisone (Rayos)

View full drug information

Prednisone is an immunosuppressant used to treat autoimmune disorders; it may decrease

inflammation by reversing increased capillary permeability and suppressing PMN activity. It
stabilizes lysosomal membranes and suppresses lymphocyte and antibody production.
Prednisolone (Millipred, Orapred, Orapred ODT, Prelone)

View full drug information

Corticosteroids act as potent inhibitors of inflammation. They may cause profound and varied
metabolic effects, particularly in relation to salt, water, and glucose tolerance, in addition to
their modification of the immune response of the body.

Cyclophosphamide

View full drug information

Cyclophosphamide

Cyclophosphamide is an alkylating agent that is chemically related to nitrogen mustards. It is

a cyclic polypeptide that suppresses some humoral activity. Cyclophosphamide is
transformed in the liver to active alkylating metabolites, which interfere with the growth of
rapidly proliferating cells. When cyclophosphamide is used in autoimmune diseases, its
mechanism of action is thought to involve immunosuppression through destruction of
immune cells by DNA cross-linking.

Analgesic Agents
Class Summary

Analgesic agents are most commonly used for the relief of mild-to-moderate pain. Although
the effects of NSAIDs in the treatment of pain tend to be patient-specific, ibuprofen usually is
the drug of choice for initial therapy; other options include flurbiprofen, ketoprofen, and
naproxen. Some analgesics (eg, acetaminophen and ibuprofen) are also effective for treating
fever.

Ibuprofen (Advil, Motrin, I-Prin, Ibu-200, Neo-Profen)

View full drug information

Ibuprofen is the drug of choice for treatment of mild-to-moderate pain, if not contraindicated;
it is also effective for treating fever. Ibuprofen inhibits inflammatory reactions and pain,
probably by decreasing the activity of cyclooxygenase, thereby inhibiting prostaglandin
synthesis.

Acetaminophen (FeverAll, Q-Pap, Tylenol, APAP 500, Acephen)

View full drug information

Acetaminophen is effective for treating fever and relieving mild-to-moderate pain. It inhibits
the action of endogenous pyrogens on heat-regulating centers; it also reduces fever by a direct
action on the hypothalamic heat-regulating centers, thereby, in turn, increasing the dissipation
of body heat via sweating and vasodilation.
Flurbiprofen

View full drug information

Flurbiprofen has analgesic, antipyretic, and anti-inflammatory effects. It may inhibit

cyclooxygenase, causing inhibition of prostaglandin biosynthesis.

Ketoprofen

View full drug information

Ketoprofen is used for relief of mild-to-moderate pain and inflammation.

Naproxen (Anaprox, Naprelan, Naprosyn, Aleve)

View full drug information

Naproxen is used for relief of mild-to-moderate pain. It inhibits inflammatory reactions and
pain by decreasing the activity of cyclooxygenase, thereby reducing prostaglandin synthesis.

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Gonzlez-Gay MA. Henoch-Schnlein purpura in adulthood and childhood: two
different expressions of the same syndrome. Arthritis Rheum. 1997 May. 40(5):859-
64. [Medline].
3. Szer IS. Henoch-Schnlein purpura. Curr Opin Rheumatol. 1994 Jan. 6(1):25-31.
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Pendahuluan
Henoch-Schnleinpurpura (diucapkan: heh-nokshoon-line purr-puh-ruh) - biasanya hanya disebut
HSP, adalah suatu kondisi yang menyebabkan pembuluh darah kecil, atau kapiler, menjadi bengkak
dan iritasi. Peradangan ini, disebut vasculitis, biasanya terjadi di kulit, usus, dan ginjal. Pembuluh
darah meradang di kulit bisa mengalami kebocoran sel darah merah, menyebabkan ruam
karakteristik yang disebut purpura. Usus dan ginjal juga bisa membengkak dan mengalami
perdarahan.
Gangguan itu dinamakan demikian setelah dua dokter Jerman, Eduard Henoch dan Johann
Schnlein, yang pertama kali menggambarkan penyakit pada tahun 1800-an. Kadang-kadang juga
disebut Purpura Alergi atau Anaphylactoid Purpura.
HSP terjadi lebih sering pada anak-anak dibandingkan pada orang dewasa, biasanya terjadi antara
usia 2 dan 11. Ini adalah salah satu bentuk yang paling umum dari vaskulitis pada anak-anak, dan
anak laki-laki mendapatkan sekitar dua kali sesering anak perempuan.

Penyebab
Meskipun tidak benar-benar diketahui apa yang menyebabkan HSP, diduga HSP terjadi ketika sistem
kekebalan tubuh tidak berfungsi sebagaimana mestinya. Protein yang disebut immunoglobulin A (IgA)
dalam pembuluh darah berperan dalam reaksi ini.
Dalam kebanyakan kasus, HSP terjadi setelah seorang anak mengalami infeksi bakteri atau virus
pada saluran pernafasan bagian atas (sinus, tenggorokan, atau paru-paru). Namun obat-obat
tertentu, reaksi makanan, gigitan serangga, dan vaksinasi juga dapat menyebabkan hal itu.
HSP tidak dapat ditularkan dari satu orang ke orang lain.

Tanda dan Gejala

Tanda-tanda umum dan gejala HSP meliputi:
Ada Trias Gejala:
1. purpura, ruam yang timbul ungu kemerahan
2. nyeri sendi dan peradangan
3. sakit perut

Diketemukan darah dalam urin atau tinja, atau gangguan ginjal berupa hematuria atau proteinuria.
Selain itu ada juga gejala:
demam
sakit kepala
Ruam terjadi di hampir semua kasus dan biasanya membantu dokter mendiagnosa HSP. Ada juga
titik-titik merah (disebut petechiae), memar, atau kadang-kadang lecet. Ruam biasanya terjadi pada
kaki dan bokong, tetapi juga bisa muncul di bagian lain dari tubuh, seperti siku, lengan, wajah, dan
badan.
Sebagian besar anak-anak dengan HSP juga mengalami nyeri sendi dan bengkak. Gejala-gejala ini
dapat terjadi sebelum ruam muncul. Paling sering terkena adalah sendi-sendi besar, seperti lutut,
pergelangan kaki, dan siku, tapi tangan dan kaki.
Sakit perut biasanya dimulai seminggu setelah ruam muncul. Nyeri intermiten dan dapat disertai
mual, muntah, atau diare. Beberapa anak akan ditemui darah dalam tinja (disebabkan oleh pembuluh
darah yang pecah), tetapi mungkin tidak terlihat.
HSP dapat mempengaruhi ginjal. Sejumlah kecil darah atau protein dapat ditemukan dalam urin,
yang kadang-kadang bisa berdarah.

Diagnosis
Ketika ada ruam yang khas pada kaki dan bokong, terutama jika disertai dengan nyeri perut atau
sendi, dokter dapat dengan mudah mendiagnosa HSP. Diagnosis mungkin lebih sulit jika nyeri sendi
atau gejala perut hadir sebelum ruam muncul, atau jika gejala butuh beberapa minggu untuk muncul.
Untuk membantu membuat diagnosis, mungkin direkomendasikan biopsi kulit.
Diperlukan meminta tes darah rutin untuk mencari tanda-tanda infeksi, anemia, atau penyakit ginjal.
Jika sakit perut parah, tes pencitraan (seperti sinar-X atau USG) mungkin diperlukan. Sebuah sampel
tinja dapat memeriksa darah dalam tinja dan urin.
Hingga setengah dari anak-anak yang menderita HSP akan memiliki komplikasi ginjal mereka,
sehingga mungkin perlu diperiksa fungsi ginjal selama beberapa bulan. Jika dicurigai HSP yang telah
menyebabkan kerusakan ginjal, anak mungkin perlu untuk kosultasi dokter ginjal (nephrologist).

Pengobatan
Gejala HSP biasanya berlangsung selama sekitar satu bulan. Sebagian besar hilang sendiri tanpa
pengobatan. Untuk membantu anak merasa lebih baik, perlu obat-obatan tertentu, seperti:
antibiotik untuk mengobati infeksi penyebab, jika terjadi infeksi.
obat penghilang rasa sakit (seperti asetaminofen)
anti-inflamasi non steroid (seperti ibuprofen) untuk meredakan nyeri sendi dan peradangan
kortikosteroid (misalnya prednisone) untuk sakit perut parah atau kelainan ginjal.
Juga, perlu berhenti minum obat tertentu jika ada kemungkinan bahwa obat itu penyebab HSP
tersebut.
Sementara di rumah, dijaga anak merasa senyaman mungkin. Pastikan anak mendapatkan banyak
cairan, istirahat dan minuman. Jika anak berhenti makan atau minum, atau sakit perut parah atau
masalah ginjal, rawat inap mungkin diperlukan.

Prognosis
Kebanyakan anak dengan HSP sepenuhnya pulih dalam waktu satu bulan dan tidak memiliki
masalah jangka panjang. Anak-anak yang ginjal nya terkena perlu ke dokter untuk pemeriksaan rutin
untuk memantau fungsi ginjal.
Sekitar sepertiga dari mereka yang menderita HSP mengalami kekambuhan lagi di masa datang,
biasanya beberapa bulan setelah episode pertama. Ini (bersama dengan masalah ginjal kronis) lebih
sering terjadi pada anak-anak yang lebih tua dan orang dewasa. Jika HSP tidak kambuh kembali,
biasanya prognosanya baik

DEFINISI
Adalah sindrom klinis yang disebabkan oleh vaskulitis pembuluh darah kecil sistemik yang ditandai
dengan lesi spesifik berupa purpura nontrombositopenik, artritis atau atralgia, nyeri abdomen atau
perdarahan gastrointestinalis, dan kadang kadang nefritis atau hematuria. Nama lain penyakit ini
adalah purpura anafilaktoid, purpura alergik dan vaskulitis alergik.

EPIDEMIOLOGI
Penyakit ini terutama terdapat pada anak umur 2 15 tahun (usia anak sekolah) dengan puncaknya
pada umur 4 7 tahun. Terdapat lebih banyak pada anak laki laki dibanding anak perempuan (1,5 :
1).

ETIOLOGI
Sampai sekarang penyebab penyakit ini belum diketahui. Diduga beberapa faktor memegang
peranan, antara lain faktor genetik, infeksi traktus respiratorius bagian atas, makanan, gigitan
serangga, paparan terhadap dingin, imunisasi ( vaksin varisela, rubella, rubeolla, hepatitis A dan B,
paratifoid A dan B, tifoid, kolera) dan obat obatan (ampisillin, eritromisin, kina, penisilin, quinidin,
quinin).

Infeksi bisa berasal dari bakteri (spesies Haemophilus, Mycoplasma, Parainfluenzae, Legionella,
Yersinia, Shigella dan Salmonella) ataupun virus (adenovirus, varisela, parvovirus, virus EpsteinBarr).
Vaskulitis juga dapat berkembang setelah terapi antireumatik, termasuk penggunan metotreksat
dan agen anti TNF (Tumor Necrosis Factor).

Namun, IgA jelas mempunyai peranan penting, ditandai dengan peningkatan konsentrasi IgA serum,
kompleks imun dan deposit IgA di dinding pembuluh darah dan mesangium renal. HSP adalah suatu
kelainan yang hampir selalu terkait dengan kelainan pada IgA1 daripada IgA2.

Berbagai kondisi yang dapat menyebabkan HSP antara lain:

Infeksi :- Mononukleosis - Infeksi Streptokokus grup A - Sirosis karena Hepatitis-C - Infeksi parvovirus
B19 - Infeksi Yersinia Hepatitis - Infeksi Mikoplasma - Virus Epstein-Barr - Infeksi viral Varizella-
zoster Vaksin :- Tifoid - Campak - Makanan - Gigitan serangga - Paparan terhadap dingin - Infeksi
Shigella - Infeksi Salmonella - Enteritis Campylobacter - Kolera - Demam kuning
Alergen - Obat (ampisillin, eritromisin, penisilin, kuinidin, kuinin)
Penyakit idiopatik : Glomerulocystic kidney disease

PATOFISIOLOGI
Dari biopsi lesi pada kulit atau ginjal, diketahui adanya deposit kompleks imun yang mengandung
IgA. Diketahui pula adanya aktivasi komplemen jalur alternatif. Deposit kompleks imun dan aktivasi
komplemen mengakibatkan aktivasi mediator inflamasi termasuk prostaglandin vaskular seperti
prostasiklin, sehingga terjadi inflamasi pada pembuluh darah kecil di kulit, ginjal, sendi dan abdomen
dan terjadi purpura di kulit, nefritis, artritis dan perdarahan gastrointestinalis.

Beberapa faktor imunologis juga diduga berperan dalam patogenesis PHS, seperti perubahan
produksi interleukin dan faktor pertumbuhan yang berperan dalam mediator inflamasi. TNF, IL-1 dan
IL-6 bisa memediasi proses inflamasi pada HSP. Meningkatnya kadar faktor pertumbuhan hepatosit
selama fase akut HSP dapat menunjukkan adanya kemungkinan kerusakan atau disfungsi sel
endotel. Meningkatnya faktor pertumbuhan endotel vaskuler dapat setidaknya menginduksi
sebagian perubahan ini.

Sitokin dianggap terlibat dalam patogenesis HSP, dan endotelin (ET), yang merupakan hormon
vasokonstriktor yang diproduksi oleh sel endotelial, juga dianggap turut berperan. Kadar ET-1 jauh
lebih besar pada fase akut penyakit ini dibanding pada fase remisi. Namun tingginya kadar ET-1 tidak
memiliki hubungan dengan tingkat morbiditas, keparahan penyakit, atau respon reaktan fase akut.

MANIFESTASI KLINIS HSP

biasanya muncul dengan trias berupa ruam purpura pada ekstremitas bawah, nyeri abdomen atau
kelainan ginjal dan artritis. Namun trias tidak selalu ada, sehingga seringkali mengarahkan kepada
diagnosis yang tidak tepat.

Gejala klinis mula mula berupa ruam makula eritomatosa pada kulit ekstremitas bawah yang
simetris yang berlanjut menjadi palpable purpura tanpa adanya trombositopenia.
Ruam awalnya terbatas pada kulit maleolus tapi biasanya kemudian akan meluas ke permukaan
dorsal kaki, bokong dan lengan bagian luar. Dalam 12 24 jam makula akan berubah menjadi lesi
purpura yang berwarna merah gelap dan memiliki diameter 0,5 2 cm. Lesi dapat menyatu menjadi
plak yang lebih besar yang menyerupai echimosis yang kemudian dapat mengalami ulserasi.

Purpura terutama terdapat pada kulit yang sering terkena tekanan (pressure-bearing surfaces).
Kelainan kulit ini ditemukan pada 100% kasus dan merupakan 50% keluhan penderita pada waktu
berobat.

Kelainan kulit dapat pula ditemukan pada wajah dan tubuh. Kelainan pada kulit dapat disertai rasa
gatal. Pada bentuk yang tidak klasik, kelainan kulit yang ada dapat berupa vesikel hingga menyerupai
eritema multiform. Kelainan akut pada kulit ini dapat berlangsung beberapa minggu dan
menghilang, tetapi dapat pula rekuren.

Edema skrotum juga dapat terjadi dan gejalanya mirip dengan torsio testis. Gejala prodromal dapat
terdiri dari demam dengan suhu tidak lebih dari 38C, nyeri kepala dan anoreksia.

Pada anak berumur kurang dari 2 tahun, gambaran klinis disa didominasi oelh edema kulit kepala,
periorbital, tangan dan kaki. Gambaran ini disebut AHEI (Acute Hemorrhagic Edema of Infancy).
Selain purpura, ditemukan pula gejala artralgia dan artritis yang cenderung bersifat migran dan
mengenai sendi besar ekstremitas bawah seperti lutut dan pergelangan kaki, namun dapat pula
mengenai pergelangan tangan, siku dan persendian di jari tangan.

Kelainan ini timbul lebih dulu (1 2 hari) dari kelainan kulit. Sendi yang terkena dapat menjadi
bengkak, nyeri dan sakit bila digerakkan, biasanya tanpa efusi, kemerahan ataupun panas. Kelainan
teutama periartrikular dan bersifat sementara, dapat pula rekuren pada masa penyakit aktif tetapi
tidak menimbulkan deformitas menetap.

Pada penyakit ini dapat ditemukan adanya gangguan abdominal berupa nyeri abdomen atau
perdarahan gastrointestinalis.

Keluhan abdomen biasanya timbul setelah timbul kelainan pada kulit (1 4 minggu setelah onset).

Organ yang paling sering terlibat adalah duodenum dan usus halus. Nyeri abdomen dapat berupa
kolik abdomen yang berat, lokasi di periumbilikal dan disertai mual, muntah, bahkan muntah darah
dan kadang kadang terdapat perforasi usus dan intususepsi ileoileal lebih sering terjadi dibanding
ileokolonal.

Intususepsi atau perforasi disebabkan oleh vaskulitis dinding usus yang menyebabkan edema dan
perdarahan submukosa dan intramural. Kadang dapat juga terjadi infark usus yang disertai perforasi
maupun tidak. Selain itu dapat juga ditemukan kelainan ginjal, meliputi hematuria, proteinuria
(<2g/d), sindrom nefrotik (proteinuria >40mg/m2/jam) atau nefritis. Penyakit pada ginjal juga
biasanya muncul 1 bulan setelah onset ruam kulit.

Adanya kelainan kulit yang persisten sampai 2 3 bulan, biasanya berhubungan dengan nefropati
atau penyakit ginjal yang berat. Resiko nefritis meningkat pada usia di atas 7 tahun, lesi purpura
persisten, keluhan abdomen yang berat dana penurunan aktivitas faktor XIII. Gangguan ginjal
biasanya ringan, meskipun beberapa ada yang menjadi kronik.
Seringkali derajat keparahan nefritis tidak berhubungan dengan parahnya gejala HSP yang lain. Pada
pasien HSP dapat timbul adanya oedem. Oedem ini tidak bergantung pada derajat proteinuria
namun lebih pada derajat vaskulitis yang terjadi. Namun oedem tersebut memang dihubungkan
dengan kejadian proteinuria pada pasien.

Kadang kadang HSP dapat disertai dengan gejala gejala gangguan sistem saraf pusat, terutama
sakit kepala. Pada HSP dapat ditemukan adanya vaskulitis serebral. Pada beberapa kasus langka, HSP
diduga dapat menyebabkan gangguan serius seperti kejang, paresis atau koma.

Gejala gejala gangguan neurologis lain yang dapat muncul antara lain perubahan tingkat
kesadaran, apatis, somnolen, hiperaktivitas, iritabilitas, ketidakstabilan emosi, kejang (parsial, parsial
kompleks, umum, status epileptikus), dan defisit neurologis fokal (afasia, ataxia, korea, hemiparesis,
paraparesis, kuadraparesis. Dapat juga terjadi poliradikuloneuropati (sindroma Guillain-Barr) dan
mononeuropati (nervus fasialis, femoralis, ulnaris).

Hati dan kandung empedu juga bisa terlibat dengan gejala hepatomegali, hidrops kandung empedu,
kolesistitis. Semua ini bisa menyebabkan keluhan nyeri abdomen pada pasien. Apendisitis akut juga
pernah dilaporkan terjadi pada pasien HSP.

Gejala - gejala lain yang pernah dilaporkan tetapi jarang terjadi antara lain vaskulitis miokardia,
vaskulitis paru yang menyebabkan perdarahan paru bilateral, ureteritis stenosis, oedem penis,
orkitis, priapisme, perdarahan intrakranial, hematoma subperiosteal orbital bilateral, hematoma
adrenal dan pankreatitis akut.

PEMERIKSAAN PENUNJANG
Pada pemeriksaan laboratorium tidak terlihat adanya kelainan spesifik. Jumlah trombosit normal
atau meningkat, membedakan purpura yang disebabkan oleh trombositopenia. Dapat terjadi
leukositosis moderat dan anemia normokromik, biasanya berhubungan dengan perdarahan
gastrointestinal. Biasanya juga terdapat eosinofilia. Laju endap darah dapat meningkat maupun
normal. Kadar komplemen seperti C1q, C3 dan C4 dapat normal maupun menurun. Pemeriksaan
kadar IgA dalam darah mungkin meningkat, demikian pula limfosit yang mengandung IgA.

Analisis urin dapat menunjukkan hematuria, proteinuria maupun penurunan kreatinin klirens
menandakan mulai adanya kerusakan ginjal atau karena dehidrasi, demikian pula pada feses dapat
ditemukan darah.

Pemeriksaan ANA dan RF biasanya negatif, faktor VII dan XIII dapat menurun.

Biopsi lesi kulit menunjukkan adanya vaskulitis leukositoklastik.

Imunofluorosensi menunjukkan adanya deposit IgA dan komplemen pada dinding pembuluh darah.

Pada pemeriksaan radiologi dapat ditemukan penurunan motilitas usus yang ditandai dengan
pelebaran lumen usus ataupun intususepsi melalui pemeriksaan barium.

Terkadang pemeriksaan barium juga dapat mengkoreksi intususepsi tersebut.

DIAGNOSIS
Diagnosis lebih banyak ditegakkan berdasarkan gejala klinis yang spesifik daripada dengan bantuan
pemeriksaan penunjang. Gejala yang dapat mengarahkan kepada diagnosis HSP yaitu ruam purpurik
pada kulit terutama di bokong dan ekstremitas bagian bawah dengan satu atau lebih gejala berikut:
nyeri abdomen atau perdarahan gastrointestinalis, artralgia atau artritis, dan hematuria atau nefritis.

Kriteria Definisi Purpura non trombositopenia (palpable Lesi kulit hemoragik yang dapat diraba,
purpura) terdapat elevasi kulit, tidak berhubungan dengan trombositopenia Usia onset 20 tahun
Onset gejala pertama 20 tahun Gejala abdominal / gangguan saluran Nyeri abdominal difus,
memberat cerna (Bowel angina) Granulosit dinding pada biopsi setelah makan atau diagnosis
iskemia usus, biasanya termasuk BAB berdarah Perubahan histologi menunjukkan granulosit pada
dinding arteriol atau venula

Untuk kepentingan klasifikasi, pasien dikatakan mempunyai HSP bila memenuhi setidaknya 2 dari
kriteria yang ada. Tabel diambil dari Buku Ajar Alergi-Imunologi Anak 2007. Diferensial diagnosis dari
HSP berdasarkan gejala yang dapat timbul antara lain akut abdomen, meningitis akibat
meningokokus, SLE, endokarditis bakterial, ITP, demam reumatik, Rocky mountain spotted fever,
reaksi alergi obat obatan, nefropati IgA, artritis reumatoid.

PENGOBATAN
Tidak ada pengobatan definitif pada penderita HSP. Pengobatan adalah suportif dan simtomatis,
meliputi pemeliharaan hidrasi, nutrisi, keseimbangan elektrolit dan mengatasi nyeri dengan
analgesik.

Untuk keluhan artritis ringan dan demam dapat digunakan OAINS seperti ibuprofen.

Dosis ibuprofen yang dapat diberikan adalah 10mg/kgBB/6 jam.

Edema dapat diatasi dengan elevasi tungkai. Selama ada keluhan muntah dan nyeri perut, diet
diberikan dalam bentuk makanan lunak. Penggunaan asam asetil salisilat harus dihindarkan, karena
dapat menyebabkan gangguan fungsi trombosit yaitu petekie dan perdarahan saluran cerna. Bila ada
gejala abdomen akut, dilakukan operasi. Bila terdapat kelainan ginjal progresif dapat diberi
kortikosteroid yang dikombinasi dengan imunosupresan. Metilprednisolon IV dapat mencegah
perburukan penyakit ginjal bila diberikan secara dini.

Dosis yang dapat digunakan adalah metilprednisolon 250 750 mg/hr IV selama 3 7 hari
dikombinasi dengan siklofosfamid 100 200 mg/hr untuk fase akut HSP yang berat. Dilanjutkan
dengan pemberian kortikosteroid (prednison 100 200 mg oral) selang sehari dan siklofosfamid 100
200 mg/hr selama 30 75 hari sebelum akhirnya siklofosfamid dihentikan langsung dan tappering-
off steroid hingga 6 bulan.

Terapi prednison dapat diberikan dengan dosis 1 2 mg/kgBB/hr secara oral, terbagi dalam 3 4
dosis selama 5 7 hari. Kortikosteroid diberikan dalam keadaan penyakit dengan gejala sangat
berat, artritis, manifestasi vaskulitis pada SSP, paru dan testis, nyeri abdomen berat, perdarahan
saluran cerna, edema dan sindrom nefrotik persisten. Pemberian dini pada fase akut dapat
mencegah perdarahan, obstruksi, intususepsi dan perforasi saluran cerna.

PROGNOSIS
Pada umumnya prognosis adalah baik, dapat sembuh secara spontan dalam beberapa hari atau
minggu (biasanya dalam 4 minggu setelah onset). Rekurensi dapat terjadi pada 50% kasus. Pada
beberapa kasus terjadi nefritis kronik, bahkan sampai menderita gagal ginjal. Bila manifestasi
awalnya berupa kelainan ginjal yang berat, maka perlu dilakukan pemantauan fungsi ginjal setiap 6
bulan hingga 2 tahun pasca sakit.

Penyulit yang dapat terjadi antara lain perdarahan saluran cerna, obstruksi, intususepsi, perforasi,
gagal ginjal akut dan gangguan neurologi. Penyulit pada saluran cerna, ginjal dan neurologi pada fase
akut dapat menimbulkan kematian, walaupun hal ini jarang terjadi.

Prognosis buruk ditandai dengan penyakit ginjal dalam 3 minggu setelah onset, eksaserbasi yang
dikaitkan dengan nefropati, penurunan aktivitas faktor XIII, hipertensi, adanya gagal ginjal dan pada
biopsi ginjal ditemukan badan kresens pada glomeruli, infiltrasi makrofag dan penyakit
tubulointerstisia

127
Volume 45 Number 3 September 2012
Henoch-Schnlein purpura in children:
its relation to oral and
its relation to oral and
relation to oral and
relation to oral and
oral and
dental health
arlette Suzy Puspa Pertiwi
Department of Pediatric Dentistry
Faculty of Dentistry, Universitas Padjadjaran
Bandung - Indonesia
abstract
Background:
Henoch-Schnlein purpura (HSP) is a rare systemic small vessel vasculitis, which commonly
occur in children
between 2 and 10 years of age. The course of the disease is often self-limiting, although may
manifest long-term renal morbidity. The
severity of renal involvement decides about the prognosis of this disease. Many factors can
trigger the disease attack, which is the most
common is bacterial invasion. Since the oral cavity is often refer as infectious foci to other part of
the body, it seemed rationally to
be part that contribute the course of disease, thus management of these infectious foci, if possible,
gives rise to an astoundingly good
prognosis.
Purpose:
This paper will describe a review on HSP and the possible association with oral and dental health
since it might
be related to the prognosis of HSP.
reviews:
Rashes in children are common; they may develop a rash after prescription of antibiotics.
Nevertheless there are some childhood diseases that may manifest a rash presentation, such as
HSP. It is important for pediatric dentist
to have knowledge about HSP and consider the possibility of dental treatment or disease as
potential triggers.
Conclusion:
Oral and
dental condition may be the trigger cause of HSP attack. Therefore, it is important for pediatric
dental practitioner to be aware of the
course of the disease in order to limit the expanding complications.
Key words:
Henoch-Schnlein purpura, infectious foci, children
abstrak
latar belakang:
Henoch-Schnlein purpura (HSP) merupakan vaskulitis pembuluh darah kecil sistemik yang
jarang terjadi dan
biasanya menyerang anak usia 2 hingga 10 tahun. Penyakit tersebut seringkali dapat sembuh
sendiri, tetapi pada jangka panjang
dapat bermanifestasi dengan morbiditas ginjal. Keparahan keterlibatan ginjal menentukan
prognosis penyakit. Banyak faktor yang
dapat memicu serangan penyakit, tersering adalah invasi bakteri. Karena rongga mulut sering kali
merupakan fokus infeksi terhadap
bagian lain dari tubuh, maka mempunyai peluang sebagai faktor pemicu timbulnya penyakit,
sehingga penatalaksanaan fokus infeksi
dalam rongga mulut, jika ada, dapat memberikan prognosis yang baik pada pasien.
tujuan:
Makalah ini akan menggambarkan
tinjauan mengenai HSP dan hubungannya dengan kesehatan gigi dan mulut berkaitan
pengaruhnya terhadap prognosis HSP.
tinjauan
Pustaka:
Ruam sering terjadi pada anak; pasien anak dapat memperlihatkan gejala ruam setelah pemberian
antibiotika. Selain itu,
beberapa penyakit dapat bermanifestasi sebagai ruam, misalnya HSP. Merupakan hal yang
penting bagi dokter gigi anak untuk memiliki
pengetahuan mengenai HSP dan mempertimbangkan perawatan atau penyakit gigi sebagai
pemicu potensial.
Kesimpulan:
Keadaan
gigi dan mulut dapat merupakan pencetus serangan HSP, oleh karena itu penting bagi dokter gigi
anak untuk memahami perjalanan
penyakit sehingga dapat membatasi komplikasi yang terjadi.
Kata kunci:
Henoch-Schnlein purpura, fokus infeksi, anak
Correspondence
: Arlette Suzy Puspa Pertiwi, c/o: Departemen Kedokteran Gigi Anak, Fakultas Kedokteran Gigi
Universitas Padjadjaran,
Jl. Sekeloa Selatan 1 Bandung, Indonesia. E-mail: arlettesuzy@yahoo.com
Literature Reviews
128
Dent. J. (Maj. Ked. Gigi), Volume 45 Number 3 September 2012: 127132
introduction
Henoch-Schonlein purpura (HSP) is the most common
type of systemic vasculitis in childhood and diagnosed
when palpable purpura is present plus one of the following:
diffuse abdominal pain, any biopsy showing predominant
immunoglobulin A (IgA) deposition, arthritis or arthralgia,
and renal involvement.
1
Symptoms can begin in children,
most commonly between the ages of 4 and 7 years, soon
after an upper respiratory tract infection or streptococcal
pharyngitis. Children may develop arthritis, leading to pain.
A rash may start as urticaria or erythematous maculopapules
on the legs and buttocks. Eventually these spots blend to
form purpura in the skin. Abdominal pain that can be quite
severe may also present in some cases. Children younger
than 2 years with HSP are more likely to develop edema
of various areas of their bodies, which is a result of leaky
small blood vessels in the skin. Kidney involvement can
also cause edema, hematuria or proteinuria.
2
Although its
cause is unknown, it is often associated with infectious
agents such as group A streptococci
3
and also as immune
complex disease since IgA, which is caused by mucosal
infections, is known to play an important role in its immune-
pathogenesis.
4
A high prevalence of infectious foci in oral
as well as ear, nose, and throat diseases was revealed in
children with HSP.
5
HSP is known to have a high probability of being
spontaneously cured if supportive treatment is the primary
intervention. However, it sometimes develops into severe
conditions with a high rate of reoccurrence. No form of
therapy has ever been shown to decrease the duration of
the disease or prevent recurrences. Nephritis is the most
serious long-term complication of HSP. Although early
aggressive therapy has been recommended for children
with such severe renal involvement, there is little evidence
to indicate the best treatment for it.
6
Since the oral cavity is often refer as infectious foci to
other part of the body, and also structured by mucous tissue,
it seemed rationally to be related to HSP. Management
of these infectious foci, if possible, gives rise to an
astoundingly good prognosis in reducing the recurrent
attacks of HSP. Tahmassebi
3
in 2007 first reported a case
of HSP following endodontic treatment. Inoue,
et al.
7
in
2008, reported that dental caries (70%) along with apical
periodontitis (53%) was found in HSP cases and conclude
that early and extensive treatment for these lesions may
prevent the complication of HSP. This paper will discuss
an overview of HSP and the possible relation of oral focal
infection, which will be valuable to pediatric dentists, since,
although HSP is generally a benign, self-limited condition,
oral and dental health of the patient might be related to the
prognosis of HSP.
Epidemiology and etiology
Henoch-Schnlein purpura (HSP) is an inflammatory
disorder characterized by a generalized vasculitis involving
the small vessels of the skin, gastrointestinal (GI) tract,
kidneys, joints, and, rarely, the lungs and central nerve
system. The syndrome takes its name from two German
physicians. In 1837, Johan Schnlein first described several
cases of peliosis rheumatica or purpura associated with
arthritis. Thirty years later, Edouard Henoch described the
GI manifestations, including vomiting, abdominal pain, and
melena. Henoch-Schnlein purpura has also been referred
to as rheumatic purpura, leukocytoclastic vasculitis, and
allergic vasculitis.
8
Although the exact cause of HSP is unknown, exposure
to various infective pathogens, drugs, vaccines, food
allergens and insect bites may be possible immunological
triggers.
9
An upper respiratory tract infection (URTI)
preceding presentation with HSP by some days or weeks
has been reported in up to 50% of cases and the occurrence
of HSP in children particularly in the autumn and winter
months suggests an infectious etiology.
911
In patients
with HSP, immunoglobulin A (IgA) immune complexes
are deposited in small vessels, as a result of exposure
to an antigen from an infection, medication, or other
environmental factors. Group A streptococci, which can
cause an upper respiratory tract infection, are the most
common pathogenic microorganisms that cause HSP.
12
Several dermatological or autoimmune diseases are thought
to correlate with odontogenic infectious diseases. For
example, Burger's disease has been linked to periodontitis,
or palmoplantar pustulosis and chronic pigmented purpura
have also been reported to have an association with an oral
focal infection.
6
HSP is considered to be associated with odontogenic
infectious diseases as well. There are a few reports that
mention the correlation between HSP and odontogenic
infectious diseases. Jinous
et al.
3
have reported a case
of HSP that had developed after endodontic treatment.
This report suggested that root canal treatment could be a
trigger for HSP, as it assumed that trepanation of the apex
may cause a streptococcal bacteremia. Environment and
microbiological flora changes in the root canal may also
cause a bacteremia. Inoue
et al
.,
7
have reported on the
efficacy of dental treatment in preventing nephropathy in
pediatric HSP. Igawa
et al
.,
13
reported that an oral focal
infection could be a precipitating factor for adult HSP,
as improvements in the skin lesions were observed after
patients being treated for the oral infection.
HSP preferentially affects children aged 211 years. The
median age is 5 years and occurs a slight predominance in
males, which is twice as females. The condition has been
reported to have an incidence of 1020 cases per 100 000
school-aged children each year.
3,11,14
More than 90 percent
of patients are children younger than 10 years, with a peak
incidence at six years of age.
However, it is also seen in
infants, adolescents, and adults. HSP is milder in infants and
children younger than two years.
Disease is more severe in
older children and adults, especially with regards to renal
involvement.
12
The true prevalence may be underestimated
because cases are often not reported. As seen in Indonesia,
the exact data about the prevalence HSP is still unclear.
129
Pertiwi:
Henoch-Schnlein purpura in children
According to secondary data in Harapan Kita Hospital
Jakarta during 6 years period (20042010), it was reported
70 cases of HSP in 216 years old children with the rate for
boys are higher (55.7%) than girls (44.3%).
15,16
Based to ethnic groups, HSP has a higher prevalence in
Caucasians and Asians than in those of African descent.
17
The disease occurs more often in the colder months and
is usually preceded by an upper respiratory infection,
particularly streptococcal, but a recent study suggests that
an occult malignancy may be the cause.
12
Despite the exact etiology of HSP remains unknown,
histologically HSP exhibits an immune mediated
leukocytoclastic vasculitis, with deposits of immunoglobulin
A (IgA) and its immune complexes within the walls of
involved vessels and organs. Patients have elevated serum
levels of IgA, IgA immune complexes, IgA anticardiolipin
antibodies, and transforming growth factor-, as well as
altered IgA glycosylation.
18
Antigen and antibody complexes, mostly IgA, form as a
result of bacterial and viral infections, vaccinations, drugs,
and autoimmune mechanisms. These antigen antibody
complexes deposit in the small vessel walls and activate
the alternate complement pathway that leads to neutrophil
accumulation resulting in inflammation and vasculitis
without a granulomatous reaction. This can involve multiple
systems including skin, gastrointestinal tract, kidney, and
joints but it can involve any organ system. Vasculitis
causes extravasation of blood and its components into the
interstitial spaces resulting in edema and hemorrhage.
19
Clinical feature
The major clinical manifestations of HSP are purpura,
arthritis, abdominal pain, gastrointestinal bleeding, and
Henoch-Schnlein purpura nephritis (HSPN).
7
The most
common clinical manifestations are illustrated in Figure
1. These can develop over days to weeks and may vary
in the order that they present. It can masquerade as many
different conditions, depending on the symptoms. Palpable
purpura and joint pain are the most common and consistent
presenting symptoms; initial diagnosis of HSP in the
absence of these symptoms may not be obvious.
18
The classic rash (Figure 2) of HSP begins as
erythematous, urticarial and macular wheals. It then
coalesces and develops into the typical ecchymoses,
petechiae, and palpable purpura. The rash occurs in 96%
cases, often manifests in a symmetrical pattern at pressure
dependent areas, such as the lower extremities and the
figure 1
.
The four classic features of Henoch-Schnlein
purpura.
18
figure 3.
Typical distribution of palpable purpura in Henoch-
Schnlein purpura.
20
figure 4.
Henoch-Schnlein purpura of the upper limb with
swollen elbow joint
.
21
figure 2.
Closer look of skin rashes.
20
130
Dent. J. (Maj. Ked. Gigi), Volume 45 Number 3 September 2012: 127132
buttocks (Figure 3). In no ambulatory children the face,
trunk, and upper extremities may be more affected. May be
urticarial and cause edema, particularly in children.
18
Joints are involved in the majority of cases, involving
lower limbs (ankles and knees) more commonly.
21
Arthralgia occurs in 84% of HSP patients and often
coexists with other symptoms.
The large joints of the
lower extremities are most commonly affected. It is none
destructive arthritis. Transient oligoarticular arthritis and
periarticular swelling may cause pain, tenderness and
restricted movement (Figure 3).
18
GI pain is often the most debilitating of the HSP
symptoms, and can be further complicated by GIT
hemorrhage (1438%), intussusception, obstruction
or perforation.
21,22
GI problems started as cramping
abdominal pain, often with vomiting, appears about a
week or more after the rash begins. Although there are
cases where GI problems occur without a rash. 25% have
GIB and 50% have occult blood loss. On endoscopy you
see pupuric lesions +/ edema, ulceration, or bowel spasm
(Figure 5).
8
Renal symptoms have a wide range of severity, from
asymptomatic microscopic hematuria, to full-blown
nephritic syndrome or nephritis. Most renal involvement
occurs early, 85% within the first month, although it can
develop later; follow-up to 6 months is recommended.
21
Begins few days to weeks after other symptoms. Urine
analysis can show proteinuria (mild), red blood cells, and
cellular casts. Many patients will be asymptomatic, but
others can develop nephritic syndrome.
8
Other symptoms are rare and usually involve the central
nervous system or lungs, from pulmonary hemorrhage
through to convulsions.
Age does play a role in the
symptomatology, with children younger than two years
showing predominantly cutaneous symptoms and signs, as
well as a much lower incidence of renal and gastrointestinal
involvement. The peak incidence is in the 46 year-old
age group with figures around 70/100 000 population,
with a very slight male predominance.
Recurrence is
relatively common and 30% of patients will have one or
more recurrences of acute vasculitis. The average duration
of disease is 4 weeks and while steroids will shorten this
period, current data suggest there is no correlation between
steroid use and in- creased frequency of relapse.
21
diagnosis, differential diagnosis and prognosis
Diagnosis of HSP depends on clinical findings and
history. It is usually not difficult if the classic triad of rash,
gastrointestinal complaints or hematuria, and arthritis is
present. When symptoms are not typical, however, the
differential diagnosis can become extensive. There is not
a specific laboratory test for the disorder, although an
elevated serum IgA level is suggestive. Some laboratory
studies can also help in excluding other diagnoses and in
evaluating renal function, including urinalysis.
8
HSP is a clinically obvious condition in the majority
of cases, but laboratory investigation would include: full
blood count,
to exclude thrombocytopenia; most often
thrombocytosis is found in HSP. Anemia may be present
but is usually an indicator of GIT hemorrhage or severe
hematuria. Renal function is obviously very important
and assists in identifying some with a rapidly progressive
glomerular disease. Erythrocyte sedimentation rate (ESR)
is elevated in approximately 60%, but is a nonspecific
inflammatory marker. IgA levels are elevated in 2550%
of patients. Albumen levels are diminished in cases of
nephritic syndrome and/or protein-losing enteropathy,
which may occur. Occult fecal blood is seen in 25%. Factor
XIII plasma levels can be measured in atypical cases and are
decreased in the majority, even prior to purpura formation.
Skin biopsy is a useful diagnostic tool in atypical cases, and
reveals a typical leucocytoclastic vasculitis with necrosis
of the vascular wall and inflammatory cell infiltrate,
accompanying IgA dermal deposition.
21
Vasculitis is not a common childhood condition
and HSP is difficult to confuse with other small-vessel
vasculitides, but a relatively short list of alternative
possibilities, which includes Kawasaki disease. Most of
these conditions can be excluded or diagnosed clinically,
but immune serological markers and a full blood count will
distinguish doubtful cases.
21
Generally the prognosis is good, with the exception
of those with significant renal involvement.
23
HSP is only
fatal in the most rare of cases. Initial attacks of HSP can last
several months, and relapses are possible. Kidney damage
related to Henoch-Schoenlein purpura is the primary cause
of morbidity and mortality. Overall, an estimated 2% of
cases progress to renal failure; as many as 20% of children
who have HSP and are treated in specialized centers require
hemodialysis. The renal prognosis appears to be worse in
adults than in children.
24
Management
Management is mainly supportive and symptomatic.
Most patients can be managed as outpatients with treatment
being directed at adequate oral hydration and pain relief.
figure 5.
Endoscopicfindingonesophagusshowing
inflammation, sub mucosal hemorrhage, and small
ulceration.
19
131
Pertiwi:
Henoch-Schnlein purpura in children
Edema of the lower extremities, buttocks, and genital area
are improved with bed rest and elevating the affected area.
18
Non-steroid anti inflammations drug also may reduce the
inflammation.
25
No effective therapeutic protocol to reduce
recurrent attacks of HSP or prevent the complication of
nephropathy has been established.
21
discussion
Although HSP is a rare inflammatory disorder of
childhood, it has clinical significance. The most serious
sequel of HSP is renal involvement. This complication
occurs in 50% of older children, but only 25% of children
younger than 2 years of age. Less than 1% of cases progress
to end-stage renal disease. Patients who develop renal
involvement generally do so within three months of the
onset of rash.
3,26,27
HSP is a self-healing benign disorder, but renal and
gastrointestinal involvement can lead to poor prognoses.
Odontogenic focus infection (OFI) as one of several
trigger cause to HSP attack need to be consider as one
of the risk factors that determine the prognosis of HSP.
OFI is a bacterial infection that tends to be overlooked by
dermatologists. Dental screening of HSP patients could help
to decrease the risk of renal and/or abdominal complications
and facilitate treatment.
13
The concept that focal infection may produce chronic
systemic diseases has now been generally accepted. Local,
septic, or mucosal infections foci anywhere in the body can
be sources of systemic diseases. To date, foci of specific
infections of the gums and the presence of abscesses around
the roots of the teeth, often unsuspected, have not received
attention in the treatment of pediatric diseases.
7
The etiological role of chronic oral infection in HSP is
supported by several other studies. The antigens of the outer
membranes of Haemophilus parainfluenzae, a common
bacterium within apical periodontitis, and antibodies against
these have been identified in the glomerular mesangium and
sera of HSP and IgA nephropathy patients.
28
In one case,
endodontic dental therapy coincidentally induced HSP.
3
Given that IgA nephropathy and HSPN are pathologically
identical diseases, all of these data suggest that chronic
infections in the oral cavity may play pathogenic roles in
HSP.
7
The high caries levels of HSP children may support
this view. Dental caries in premature teeth easily invade
through infected root canals into surrounding bony tissues,
forming apical periodontitis.
29
The most commonly identified OFI in HSP patients
was apical periodontitis in association with dental caries.
Although both are infectious diseases by nature, apical
periodontitis, which is mostly initiated from dental caries
by oral bacteria invading through infected root canals, is
a much more complex disorder in regard to infection as
well as inflammation. A thousand billion bacteria colonize
a single lesion, and more than 300 species of aerobic and
anaerobic bacteria can be isolated. Within the associated
lesions, various inflammatory cytokines are produced by
cellular components of the periapical lesion, resulting in
the persistence of active immune reactions.
7
Many degraded bacterial products and the decomposition
products of pulp tissue stagnate there. Meanwhile, bacteria
and their toxic derivatives and destroyed peripheral
tissues may egress through the apical foramen and be
captured continuously within tonsils through their surface
epithelium.
30
However, the innate secretory IgA-mediated
oral mucosal defense system may fail to eliminate bacterial
antigens owing to the presence of a tremendous amount of
bacteria. On the other hand, bacterial pathogens may enter
the blood stream during transient bacteremia, damaging the
inside smooth lining of the blood vessel walls. Collectively,
chronic and long-standing apical periodontitis have the
potential to trigger HSP.
7
It is concluded that HSP is the most common systemic
vasculitis primarily affecting children aged 315 years.
HSP is characterized by palpable purpura without
thrombocytopenia or coagulopathy, arthritis or arthralgia,
abdominal pain, and renal disease. Diagnosis depends on
clinical manifestations and no single diagnostic test can
confirm the disease.
Management is mainly supportive
and symptomatic and can usually occur in the ambulatory
setting.
Oral and dental condition may be the trigger
cause of HSP attack. Therefore, it is important for dental
practitioner to be aware of the course of the disease in order
to limit the expanding complications.
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Efficacy of early dental and ENT therapy in preventing nephropathy
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61822.
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Fam Psychians 2009; 80(7): 698704.
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Schnlein purpura in adults with odontogenic focal infection. Int J
Dermatol 2011; 50(3): 2779.
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vasculitis in children in the Czech Republic: 2-year prospective
epidemiology survey. J Rhematol 2004; 31(11): 22959.
15. Widjayanti M. Manifestasi dan komplikasi Gastrointestinal pada
Purpura Henoch Schonlein. Sari Pediatri 2012; 13(2): 3349.
16. Lestari E. Manifestasi renal pada anak dengan purpura Henoch
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Schoenlein. Sari Pediatri 2012; 14(1): 369.
17. Gardner-Medwin J, Dolezalova P, Cummins C, Southwood T.
Incidence of Henoch-Schonlein purpura, Kawasaki disease, and
rare vasculitides in children of different ethnic origins. Lancet 2002;
360: 1197.
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Australian Family Physician 2009; 38(5): 3214.
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purpuraa case report and review of the literature. Gastroenterology
Research and Practice 2010: 17.
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Henoch-Schonlein purpura. Dental Update 2009; 36(2): 124.
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& Clinical Immunology 2010; 23(3): 11620.
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Setiabudiawan B, Ghrani R, Sapartini G, Sari N, Garna H. Bula
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Pediatri 2011; 13(4): 25764.
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Watson L, Richardson A, Holt R, Jones C, Beresford M. Henoch
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Sari Pediatri 2005; 7(1): 459.
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Sugiyama H, Watanabe N, Onoda T, Kikumoto Y, Yamamoto M,
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Schnlein purpura nephritis with tonsillectomy and steroid pulse
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Ogura Y, Suzuki S, Shirakawa T. Haemophilus parainfluenzae
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Schnlein nephritis. Am J Kidney Dis 2000; 36(1): 4752.
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Inouea C, Matsutanib S, Ishidoyab M, Hommab R, Chibaa Y,
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ACTA Universitatis Ouluensis, Medical; 2012
Henoch-Schonlein Purpura

DEFINISI:
Adalah suatu sindrom klinis yang disebabkan oleh vaskulitis sistemik pembuluh darah kecil ditandai
oleh lesi spesifik nontrombositopenik purpura, arthritis atau arthralgia, sakit perut atau perdarahan
gastrointestinal, dan kadang-kadang nefritis atau hematuria. Nama lain penyakit ini adalah purpura
anafilaktoid, purpura alergi dan vaskulitis alergi.

EPIDEMIOLOGI:
Penyakit ini terutama ditemukan pada anak usia 2-15 tahun (anak usia sekolah) dengan puncak pada
usia 4-7 tahun. Ada lebih pada anak laki-laki dibandingkan anak perempuan (1,5: 1).

Etiologi:
Sampai saat ini penyebab penyakit ini tidak diketahui. Beberapa faktor diduga berperan, antara lain
faktor genetik, infeksi saluran pernapasan atas, makanan, gigitan serangga, paparan dingin,
imunisasi (vaksin varicella, rubella, rubeolla, hepatitis A dan B, paratifoid A dan B, tipus, kolera) dan
antibiotik (ampisillin, eritromisin, kina, penisilin, quinidin, kina). Infeksi dapat disebabkan oleh
bakteri (spesies Haemophilus, Mycoplasma, Parainfluenzae, Legionella, Yersinia, Shigella dan
Salmonella) atau virus (adenovirus, varisela, parvovirus, virus Epstein-Barr). Vaskulitis juga dapat
mengembangkan setelah terapi antireumatik, termasuk penggunaan metotreksat dan anti-TNF agen
(Tumor Necrosis Factor). Namun, IgA jelas memiliki peran penting, ditandai oleh meningkatnya
konsentrasi serum IgA, kompleks imun IgA dan deposito di dinding pembuluh darah dan mesangium
ginjal. HSP adalah gangguan yang hampir selalu terkait dengan kelainan dalam IgA1 dari IgA2.

PATOFISIOLOGI:
Dari biopsi dari lesi pada kulit atau ginjal, ada endapan kompleks imun dikenal mengandung IgA.
Perhatikan juga adanya aktivasi komplemen jalur alternatif. Deposit kompleks imun dan aktivasi
komplemen mengakibatkan aktivasi mediator inflamasi termasuk prostaglandin vaskular seperti
prostasiklin, menyebabkan peradangan di pembuluh darah kecil di kulit, ginjal, sendi dan perut dan
terjadi pada kulit purpura, nefritis, arthritis dan perdarahan gastrointestinal.

KLINIS:
HSP biasanya muncul dengan trias ruam purpura pada ekstremitas bawah, nyeri perut atau
gangguan ginjal dan radang sendi. Tapi tiga serangkai tidak selalu ada, sehingga sering menyebabkan
diagnosa yang salah. Gejala klinis awal - awal bentuk ruam makula eritomatosa pada kulit dari
ekstremitas bawah yang terus menjadi purpura trombositopenia teraba simetris tanpa. Ruam
awalnya terbatas pada kulit tetapi biasanya maleolus kemudian akan diperluas ke permukaan dorsal,
bokong kaki dan lengan bagian luar. Dalam 12-24 jam lesi purpura makula akan berubah menjadi
merah gelap dan memiliki diameter 0,5 sampai 2 cm. Lesi dapat bergabung menjadi plak lebih besar
yang menyerupai echimosis yang kemudian dapat mengalami ulserasi.
Purpura terutama ditemukan pada kulit yang sering terkena tekanan (tekanan-bantalan permukaan).
Gangguan kulit ini ditemukan pada 100% kasus dan mewakili 50% dari pasien keluhan pada saat
pengobatan. Gangguan kulit juga dapat ditemukan pada wajah dan tubuh. Kelainan kulit bisa gatal.
Dalam bentuk yang tidak klasik, gangguan kulit yang ada dapat membentuk vesikel menyerupai
eritema ragam. Gangguan akut pada kulit dapat berlangsung beberapa minggu dan menghilang,
tetapi juga dapat berulang. Edema juga dapat terjadi skrotum dan gejala yang mirip dengan torsi
testis. Gejala prodromal bisa berupa demam dengan suhu tidak lebih dari 38 C sakit kepala, dan
anoreksia.
HSP biasanya muncul dengan trias ruam purpura pada ekstremitas bawah, nyeri perut atau
gangguan ginjal dan radang sendi. Tapi tiga serangkai tidak selalu ada, sehingga sering menyebabkan
diagnosa yang salah.

HENOCH-Schonlein:
Gejala klinis awal - awal bentuk ruam makula eritomatosa pada kulit dari ekstremitas bawah yang
terus menjadi purpura trombositopenia teraba simetris tanpa. Ruam awalnya terbatas pada kulit
tetapi biasanya maleolus kemudian akan diperluas ke permukaan dorsal, bokong kaki dan lengan
bagian luar. Dalam 12-24 jam lesi purpura makula akan berubah menjadi merah gelap dan memiliki
diameter 0,5 sampai 2 cm. Lesi dapat bergabung menjadi plak lebih besar yang menyerupai
echimosis yang kemudian dapat mengalami ulserasi.
Purpura terutama ditemukan pada kulit yang sering terkena tekanan (tekanan-bantalan permukaan).
Gangguan kulit ini ditemukan pada 100% mewakili 50% dari kasus dan keluhan dari pasien pada saat
pengobatan. Gangguan kulit juga dapat ditemukan pada wajah dan tubuh. Kelainan kulit bisa gatal.
Dalam bentuk yang tidak klasik, gangguan kulit yang ada dapat membentuk vesikel menyerupai
eritema ragam. Gangguan akut pada kulit dapat berlangsung beberapa minggu dan menghilang,
tetapi juga dapat berulang. Edema juga dapat terjadi skrotum dan gejala yang mirip dengan torsi
testis. Gejala prodromal bisa berupa demam dengan suhu tidak lebih dari 38 C sakit kepala, dan
anoreksia.
Pada anak-anak berusia kurang dari 2 tahun, gambaran klinis didominasi oelh keberdosaan edema
kulit kepala, periorbital, tangan dan kaki. Gambar ini disebut AHEI (Edema Dengue akut Bayi).
Selain purpura, juga menemukan bahwa gejala artralgia dan artritis cenderung migran dan sekitar
sendi ekstremitas bawah, seperti lutut dan pergelangan kaki, tapi bisa juga sendi pada pergelangan
tangan, siku dan jari di tangan. Gangguan ini muncul pertama (1-2 hari) dari gangguan kulit. Sendi
yang terkena mungkin menjadi bengkak, nyeri dan sakit saat digerakkan, biasanya tanpa efusi,
kemerahan atau panas. Kelainan teutama periartrikular dan sementara, juga dapat berulang selama
penyakit aktif tetapi tidak menyebabkan cacat permanen.
Pada penyakit ini dapat ditemukan dalam bentuk nyeri perut atau perut tidak nyaman perdarahan
gastrointestinal. Keluhan perut biasanya timbul setelah terjadinya gangguan kulit (1-4 minggu
setelah onset). Organ yang paling sering terlibat adalah duodenum dan usus kecil. Nyeri perut dapat
kolik perut yang parah, lokasi di periumbilikal dan disertai oleh mual, muntah darah, muntah dan
bahkan kadang-kadang ada perforasi usus dan intususepsi lebih umum daripada ileokolonal ileoileal.
Intususepsi atau perforasi yang disebabkan oleh vaskulitis yang menyebabkan edema dinding usus
dan perdarahan submukosa dan intramural. Kadang-kadang juga dapat terjadi dengan infark usus
berlubang atau tidak.
Selain itu, juga dapat ditemukan kelainan ginjal, termasuk hematuria, proteinuria (<2g / d), sindrom
nefrotik (proteinuria> 40mg/m2 / jam) atau nefritis. Penyakit ginjal juga biasanya muncul 1 bulan
setelah timbulnya ruam kulit. Adanya gangguan kulit persisten sampai 2-3 bulan, biasanya
berhubungan dengan nefropati atau penyakit ginjal berat. Peningkatan risiko nefritis pada usia 7
tahun, gigih lesi purpura, keluhan perut parah dana penurunan aktivitas faktor XIII. Gangguan ginjal
biasanya ringan, meskipun beberapa akan menjadi kronis. Seringkali keparahan nefritis tidak
berhubungan dengan tingkat keparahan gejala lain dari HSP. Pada pasien dengan HSP mungkin
timbul edema. Edema tidak tergantung pada derajat proteinuria tetapi lebih pada tingkat vaskulitis
yang terjadi. Namun, edema memang terkait dengan insiden proteinuria pada pasien.
Kadang-kadang, HSP dapat disertai dengan gejala gangguan sistem saraf pusat, terutama sakit
kepala. HSP dapat ditemukan pada keberadaan vaskulitis serebral. Dalam beberapa kasus yang
jarang, HSP diduga dapat menyebabkan gangguan serius seperti kejang, paresis, atau koma. Gejala-
gejala gangguan neurologis lainnya yang mungkin timbul, antara lain, perubahan tingkat kesadaran,
apatis, mengantuk, hiperaktif, iritabilitas, ketidakstabilan emosi, kejang (parsial, parsial kompleks,
umum, status epileptikus), dan defisit neurologis fokal (afasia , ataksia, khorea, hemiparese,
paraparese, quadraparese. Hal ini juga dapat terjadi poliradikuloneuropathy (sindrom Guillain-Barr)
dan mononeuropati (saraf wajah, femoralis, ulnaris).
Hati dan empedu gejala kandung kemih juga mungkin terlibat dengan hepatomegali, hidrops
kandung empedu, kolesistitis. Semua ini dapat menyebabkan keluhan nyeri perut pada pasien.
Apendisitis akut juga telah dilaporkan pada pasien dengan HSP.
Gejala - gejala lain yang telah dilaporkan tetapi langka di antara vaskulitis miokardia lainnya,
vaskulitis paru yang menyebabkan perdarahan paru bilateral, stenosis ureteritis, edema penis,
orkitis, priapism, perdarahan intrakranial, hematoma orbital subperiosteal hematoma adrenal
bilateral dan pankreatitis akut.

PEMERIKSAAN PENUNJANG:
Dalam tes laboratorium tidak ada kelainan yang spesifik. Normal atau meningkat jumlah trombosit,
purpura trombositopenia yang disebabkan oleh membedakan. Dapat terjadi leukositosis moderat
dan normokromik anemia, biasanya berhubungan dengan perdarahan gastrointestinal. Biasanya ada
juga eosinofilia. Tingkat sedimentasi eritrosit dapat ditingkatkan atau normal. Tingkat melengkapi
seperti C1q, C3 dan C4 mungkin normal atau menurun. Pemeriksaan kadar IgA dalam darah dapat
meningkat, begitu pula limfosit yang mengandung IgA. Urinalisis dapat menunjukkan hematuria,
proteinuria dan penurunan bersihan kreatinin tanda awal kerusakan ginjal atau karena dehidrasi,
serta darah dalam tinja dapat ditemukan. ANA dan RF pemeriksaan biasanya negatif, faktor VII dan
XIII dapat menurun.
Biopsi lesi kulit menunjukkan vaskulitis leukositoklastik. Imunofluoresensi menunjukkan deposit IgA
dan komplemen pada dinding pembuluh darah. Pada pemeriksaan radiologis dapat ditemukan
penurunan motilitas usus ditandai dengan pelebaran lumen usus atau intususepsi dengan
pemeriksaan barium. Kadang-kadang barium juga dapat memperbaiki intususepsi.

Diagnosis:
Diagnosa ditegakkan dengan gejala klinis yang lebih khusus daripada dengan bantuan investigasi.
Gejala yang dapat mengarah pada diagnosis HSP purpurik ruam pada kulit terutama di bagian
bokong dan ekstremitas yang lebih rendah dengan satu atau lebih dari gejala berikut: nyeri perut
atau perdarahan gastrointestinal, arthralgia atau arthritis, dan hematuria atau nefritis.
Diferensial diagnosis berdasarkan gejala HSP yang mungkin timbul, antara lain, perut akut, karena
meningitis meningokokus, lupus, endokarditis bakteri, ITP, demam rematik, Rocky Mountain melihat
demam, reaksi alergi terhadap obat - obat, nefropati IgA, rheumatoid arthritis.

PENGOBATAN:
Tidak ada pengobatan definitif pada pasien dengan HSP. Pengobatan suportif dan simtomatik,
termasuk pemeliharaan hidrasi, keseimbangan gizi, elektrolit dan mengatasi rasa sakit dengan
analgesik. Untuk keluhan artritis ringan dan demam dapat digunakan NSAID seperti ibuprofen. Dosis
ibuprofen dapat diberikan 10mg/kgweight/6 jam. Edema dapat diobati dengan ketinggian kaki.
Selama ada keluhan muntah dan nyeri perut, makanan diberikan dalam bentuk makanan lunak.
Penggunaan asam salisilat asetil harus dihindari, karena dapat mengakibatkan gangguan fungsi
trombosit adalah perdarahan petechiae dan gastrointestinal. Bila ada gejala abdomen akut,
dilakukan operasi. Jika ada gangguan ginjal progresif dapat diberikan dalam kombinasi dengan
kortikosteroid imunosupresan. IV metilprednisolon dapat mencegah memburuknya penyakit ginjal
ketika diberikan dini. Dosis yang bisa digunakan adalah metilprednisolon 250-750 mg / hari IV untuk
3-7 hari dalam kombinasi dengan siklofosfamid 100-200 mg / jam untuk fase akut parah HSP.
Dilanjutkan dengan kortikosteroid (prednisone 100-200 mg secara oral) dan selang siklofosfamid
sehari 100-200 mg / jam selama 30-75 hari sebelum siklofosfamid dihentikan segera dantappering-
off steroid selama 6 bulan.
Terapi prednison dapat diberikan pada dosis 1-2 mg / kgweight / hari secara oral, dibagi dalam 3-4
dosis selama 5-7 hari. Kortikosteroid diberikan dalam keadaan penyakit dengan gejala yang sangat
berat, artritis, vaskulitis paru-paru manifestasi SSP, dan testis, nyeri perut yang parah, perdarahan
gastrointestinal, sindrom nefrotik edema dan persisten. Memberikan awal pada fase akut dapat
mencegah perdarahan, obstruksi, intususepsi dan perforasi gastrointestinal