See discussions, stats, and author profiles for this publication at: https://www.researchgate.

net/publication/6127285

Henoch-Schönlein Purpura: A Review Article

Article  in  Southern Medical Journal · September 2007

DOI: 10.1097/SMJ.0b013e3180f62d0f · Source: PubMed

CITATIONS READS
58 3,449

6 authors, including:

Paul Roberts Thomas Waller

Mayo Foundation for Medical Education and Research Mayo Foundation for Medical Education and Research
3 PUBLICATIONS   71 CITATIONS    6 PUBLICATIONS   74 CITATIONS   

SEE PROFILE SEE PROFILE

Jerry Sayre
Mayo Clinic
7 PUBLICATIONS   83 CITATIONS   

SEE PROFILE

All content following this page was uploaded by Thomas Waller on 23 August 2018.

The user has requested enhancement of the downloaded file.

 Review Article
Henoch-Schönlein Purpura: A Review Article
Paul F. Roberts, MD, Thomas A. Waller, MD, Todd M. Brinker,
Jerry W. Sayre, MD, and Robert L. Bratton, MD
Abstract: Henoch-Schönlein purpura (HSP) is the most common
vasculitis of childhood. Although HSP is typically a disease of chil-
dren, adult cases have been described. HSP can affect multiple or-
gans with a characteristic rash present in all patients. Most cases
resolve with symptomatic treatment, but serious complications can
occur such as renal failure. Primary care physicians should be well
aware of the disease because the true incidence is probably under-
estimated.
Key Words: children, Henoch-Schönlein purpura, rash, renal dis-
ease
H enoch-Schönlein purpura (HSP), a vasculitis of the small
vessels, is the most common vasculitis of childhood.
First described in 1801 by William Heberden,1,2 it was orig-
inally known as Heberden-Willan disease. In 1837, Johann
Lukas Schönlein recognized the association between purpura
and arthritis.1,3 Edouard Heinrich Henoch later reported a
case that also included abdominal pain, bloody diarrhea, and
renal involvement.1,2 Many believe that HSP is what led to
Wolfgang Amadeus Mozart’s death in 1791,4,5 although this
supposition cannot be confirmed.
The cause of HSP is unclear; however, it may occur after
an upper respiratory infection. HSP is characterized by non-
thrombocytopenic palpable purpura, abdominal pain, arthri-
tis, and glomerulonephritis. It is treated with supportive care
and is usually self-limited, although it can cause glomerulo-
nephritis and death. The prognosis is excellent in those cases
without renal disease.
Epidemiology
HSP is typically a disease of children between the ages of
3 and 10 years. Although adult cases have been described,
50% of all cases occur at or before the age of 5 years.6,7
Males are affected twice as often as females.7,8 In North
From the Department of Family Medicine, Mayo Clinic, Jacksonville, Flor-
ida; and the Department of Family Medicine, Mayo Clinic, Scottsdale,
Arizona.
Reprint requests to Dr. Paul F. Roberts, Mayo Clinic, Department of Family
Medicine, 4500 San Pablo Road, Jacksonville, FL 32224. Email:
roberts.paul@mayo.edu
Accepted March 20, 2007.
Copyright © 2007 by The Southern Medical Association
0038-4348/0⫺2000/10000-0821
Southern Medical Journal • Volume 100, Number 8, August 2007
MD, Izabela Z. Riffe, MD,
America, Caucasians have the highest incidence, and African
Americans have the lowest incidence. Although the cause of
HSP is unknown, it commonly follows an upper respiratory
tract infection. As a result, the disease is more common in
January through March.7,9
The overall incidence in children has been estimated to
be 13.5 cases per 100,000.10 In 2002, a survey was published
on the frequency and ethnic variation of childhood vasculitic
syndromes. The survey had been sent monthly to subspecial-
ists and family physicians in the United Kingdom for 3 years.
The survey results revealed that the HSP incidence was higher
than previously expected, at 22.1 cases per 100,000 popula-
tion.11 The true incidence is probably underestimated because
cases are often not reported to public health agencies.
Pathophysiology
HSP is a systemic vasculitis characterized by the tissue
deposition of IgA-containing immune complexes. The depo-
sition of IgA and occasionally IgG in the mesangial region of
the kidney is the prominent feature by fluorescence micros-
copy.12,13 On histologic examination, the appearance of renal
lesions varies from mild focal mesangial proliferation to cres-
centic glomerulonephritis.14 The pathogenesis of this disease
is similar to that of IgA nephropathy, which has the same
histologic findings in the kidney.13,15
HSP has a prominent cutaneous component with skin
lesions consisting of subepidermal hemorrhages and necro-
tizing vasculitis of the small vessels of the dermis.16 IgA is
also present in these vessels. The vasculitis can also occur in
other organs, such as the gastrointestinal tract.
Clinical Manifestations
HSP usually presents with a classic tetrad of rash, pol-
yarthralgias, abdominal pain, and renal disease.2,7 The rash,
Key Points
• Henoch-Schönlein purpura (HSP) is the most com-
mon vasculitis of childhood.
• HSP can affect multiple organs, with a rash present in
all cases.
• Treatment of HSP is usually symptomatic, but serious
complications can occur.
• The true incidence of HSP is probably underestimated.
821
Roberts et al • Henoch-Schönlein Purpura

Fig. Palpable purpura on the legs

of a child.

which occurs in all patients, is characterized clinically as Diagnosis

palpable purpura (Fig.). The lesions are typically nonblanch-
ing, as they represent extravasation of blood into the skin,14
and they often occur in groups that can persist for 3 to 10
days. They can occur anywhere on the skin but are often
concentrated on the lower legs and arms. In children, local
angioedema may precede the development of the purpura.
The polyarthralgias are present in more than 80% of patients.
They most commonly affect the knees and ankles and are
often associated with edema. These findings resolve after a
few days and leave no permanent damage.16 The abdominal
pain, occurring in more than half of the patients, is often
colicky-type pain. It frequently develops within 8 days of the
appearance of the rash. The pain is usually associated with
nausea, vomiting, and diarrhea, or perhaps constipation, and
blood and mucus are frequently present with stool passage. A
rare complication is intussusception.14,17 The renal disease,
which occurs in about 40 to 50% of patients, often presents as
mild glomerulonephritis. This produces proteinuria, micro-
scopic hematuria, and often red blood cell casts. Fortunately,
this renal involvement usually resolves spontaneously.13,16
However, a progressive renal condition may develop, and
those with persistent proteinuria will likely have worsening
renal damage.13,18 Renal failure is the most common cause of
death in patients who die with HSP.
The presence of palpable purpura with the other compo-
nents of the tetrad makes the diagnosis straightforward. How-
ever, other systemic autoimmune diseases must be consid-
ered, such as other forms of vasculitis or juvenile rheumatoid
arthritis. Few laboratory tests are useful for diagnosis. Often
mild leukocytosis with a normal platelet count is found. Oc-
casionally, eosinophilia is present. In some studies, throat
swabs for group A hemolytic streptococcus were positive in
more than 50% of patients.12,19 However, other studies have
found no increased incidence of group A hemolytic strepto-
coccus infection in children with HSP.12 Elevation of IgA in
the blood occurs in 50% of patients, and a definitive diagno-
sis is confirmed by a biopsy specimen of the skin or kidney
that shows IgA deposition.7,20
In 1990, the American College of Rheumatology devel-
oped criteria for the diagnosis of HSP21 (Table 1). According
to the criteria and for the purpose of classification, a patient
is said to have HSP if at least 2 of the 4 criteria are present.
The presence of 2 or more of the criteria yields a sensitivity
of 87.1% and a specificity of 87.7%.21
Treatment
The acute, active phase of HSP resolves spontaneously in
94% of children and 89% of adults,6 and the primary goal of

Table 1. American College of Rheumatology criteria for classification of Henoch-Schönlein purpura

Criterion Definition
1. Palpable purpura Slightly raised “palpable” hemorrhagic skin lesions; not related to thrombocytopenia
2. Age ⱕ20 years at disease onset Patient age ⱕ20 years at onset of symptoms
3. Bowel angina Diffuse abdominal pain, worse after meals, or the diagnosis of bowel ischemia, usually including bloody diarrhea
4. Wall granulocytes on biopsy Histologic changes showing granulocytes in the walls of arterioles and venules

From Mills et al.3 Used with permission.

822 © 2007 Southern Medical Association

 Review Article

Table 2. Suggested management of various manifestations of Henoch-Schönlein purpura

Short-term Corticosteroids plus
Supportive oral IV IV pulse immunosuppressive
HSP manifestation care only NSAIDs corticosteroids corticosteroids corticosteroids drugs Plasmapheresis

Mild symptoms
Rash and arthritis
Rash and mild edema
Rash and severe edema
Severe colicky abdominal
pain
Any abdominal pain with
nausea and vomiting
Scrotal or testicular
involvement
Nephrotic range proteinuria
Rapidly progressive
nephritis
Pulmonary hemorrhage
X
X X
X
X X
X X
X X
X X
X X
X X X
X X X

From Gedalia.7 Used with permission.

HSP, Henoch-Schönlein purpura; IV, intravenous; NSAID, nonsteroidal anti-inflammatory drug.

the patient’s physician is to reassure the patient and the pa-
tient’s parents of the benign nature of the disease and to
provide symptomatic treatment for the patient. The patient
should be monitored for rare but more serious complications,
such as hemorrhagic involvement of the renal, pulmonary,
gastrointestinal, genitourinary, and central nervous systems
or the joints, which usually occurs within 4 weeks of initial
presentation but may occur as late as 8 weeks.16 Serious renal
complications necessitate referral to a pediatric nephrologist.
The patient (especially a child) may need to be hospitalized
for acute monitoring purposes and hydration. Milder cases
may be managed at home if the physician has a good rela-
tionship with the patient’s parents, and follow-up is assured.
Joint pain and painful soft tissue edema usually respond to
acetaminophen or nonsteroidal anti-inflammatory drugs,22 but
oral prednisone, 1 to 2 mg/kg per day, may be necessary to
hasten their resolution.16 Anti-inflammatory agents should be
avoided in patients with extensive renal involvement. Several
anecdotal reports suggest dapsone may hasten the resolution
of the palpable rash.16
No placebo-controlled prospective studies have been con-
ducted on the use of corticosteroids in treating HSP abdom-
inal pain; however, a few retrospective analyses and multiple
case reports suggest that corticosteroids may lead to rapid
resolution of abdominal pain within 24 hours without serious
complications.3,10 In addition, a few studies have suggested
factor XIII replacement therapy to treat severe gastrointesti-
nal tract bleeding complications.16
Although most physicians who treat children agree on
the acute treatment of HSP, the treatment of the pulmonary,
neurologic, and renal complications remains controversial.23
Renal involvement is common in patients with HSP, but the
majority of patients maintain normal renal function.13 Corti-
costeroid therapy does not prevent the development of pro-
gressive renal disease.3,16 If there is evidence of marked he-
maturia with proteinuria and deterioration of renal function,
then consultation with a pediatric nephrologist is necessary.
Renal biopsy may be necessary to predict prognosis and guide
therapy.3,10,16 Crescentic nephritis discovered by renal biopsy
has a poor prognosis, with 1% of affected patients progress-
ing to end-stage renal disease.16 Treatment options for severe
renal involvement by HSP include the following:
• high-dose corticosteroids, either alone or combined
with immunosuppressive agents such as azathioprine,
cyclophosphamide, or cyclosporine;
• high-dose IV immunoglobulins;
• plasma exchange or plasmapheresis;
• corticosteroids combined with urokinase and warfarin;
• renal transplant.3,10,16
The suggested management of the various manifestations
of HSP is summarized in Table 2.3
Thirty percent of patients who recover from HSP may
have recurrent symptoms as late as 7 years after the acute
phase, and those with renal involvement may have lifelong
problems. The patient’s physician, therefore, needs to moni-
tor for complications.16
Conclusion
HSP is the most common vasculitis of childhood, and the
true incidence is probably underestimated because the num-
ber of cases is underreported. HSP can affect multiple organ
systems, and the characteristic palpable purpuric rash is
present in all patients. The vast majority of cases resolve
spontaneously, but the patients should be monitored for rare
but serious complications.
References
1. Matteson EL. Notes on the history of eponymic idiopathic vasculitis: the
diseases of Henoch and Schönlein, Wegener, Churg and Strauss, Horton,
Takayasu, Behçet and Kawasaki. Arthritis Care Res 2000;13:237–245.

Southern Medical Journal • Volume 100, Number 8, August 2007 823

Roberts et al • Henoch-Schönlein Purpura
2. Ballinger S. Henoch-Schönlein purpura. Curr Opin Rheumatol 2003;15:
591–594.
3. Mills JA, Michel BA, Bloch DA, et al. The American College of Rheu-
matology 1990 criteria for the classification of Henoch-Schönlein pur-
pura. Arthritis Rheum 1990;33:1114–1121.
4. Davies PJ. Mozart’s death: a rebuttal of Karhausen: further evidence for
Schönlein-Henoch syndrome. J R Soc Med 1991;84:737–740.
5. Kubba AK, Young M. Wolfgang Amadeus Mozart: a case report. J R
Coll Surg Edinb 1996;41:44–47.
6. Blanco R, Martinez-Taboada VM, Rodriguez-Valverde V, et al. He-
noch-Schönlein purpura in adulthood and childhood: two different ex-
pressions of the same syndrome. Arthritis Rheum 1997;40:859–864.
7. Gedalia A. Henoch-Schönlein purpura. Curr Rheumatol Rep 2004;6:
195–202.
8. Agraharkar M, Gokhale S, Le L, et al. Cardiopulmonary manifestations
of Henoch-Schönlein purpura. Am J Kidney Dis 2000;35:319–322.
9. Robson WL, Leung AK. Henoch-Schönlein purpura. Adv Pediatr 1994;
41:163–194.
10. Szer IS. Henoch-Schönlein purpura. Curr Opin Rheumatol 1994;6:25–31.
11. Gardner-Medwin JM, Dolezalova P, Cummins C, et al. Incidence of
Henoch-Schönlein purpura, Kawasaki disease, and rare vasculitides in
children of different ethnic origins. Lancet 2002;360:1197–1202.
12. Saulsbury FT. Epidemiology of Henoch-Schönlein purpura. Cleveland
Clin J Med 2002;69:SII-87–SII-9.
We make a living by what we get, we make a life by
what we give.
824
View publication stats
13. Fervenza FC. Henoch-Schönlein purpura nephritis. Int J Dermatol 2003;
42:170–177.
14. Saulsbury FT. Henoch-Schönlein purpura. Curr Opin Rheumatol 2001;
13:35–40.
15. Cotran RS, Kumar V, Collins T, editors. Robbins pathologic basis of
disease, 6th ed. Philadelphia, Saunders, 1999.
16. Rostoker G. Schönlein-Henoch purpura in children and adults: diagno-
sis, pathophysiology and management. BioDrugs 2001;15:99–138.
17. Leung AK, Chan KW. Evaluating the child with purpura. Am Fam Physi-
cian. 2001;64:419–428. Erratum in: Am Fam Physician 2002;65:1751.
18. Saulsbury FT. Henoch-Schönlein purpura in children: report of 100
patients and review of the literature. Medicine (Baltimore) 1999;78:395–
409.
19. Choong CK, Beasley SW. Intra-abdominal manifestations of Henoch-
Schönlein purpura. J Paediatr Child Health 1998;34:405–409.
20. Halling SF, Soderberg MP, Berg UB. Henoch Schönlein nephritis: clin-
ical findings related to renal function and morphology. Pediatr Nephrol
2005;20:46–51. Epub 2004 Oct 22.
21. Bailey M, Chapin W, Licht H, et al. The effects of vasculitis on the gas-
trointestinal tract and liver. Gastroenterol Clin North Am 1998;27:747–782.
22. Giangiacomo J, Tsai CC. Dermal and glomerular deposition of IgA in
anaphylactoid purpura. Am J Dis Child 1977;131:981–983.
23. Kraft DM, Mckee D, Scott C. Henoch-Schönlein purpura: a review. Am
Fam Physician 1998;58:405–408.
—Sir Winston Churchill
© 2007 Southern Medical Association