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Henoch-Schonlein Purpura Clinical

Presentation
Updated: Nov 10, 2016
Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: Craig B Langman, MD more...

PRESENTATION

History
Henoch-Schönlein 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.

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Characteristic rash of Henoch-Schönlein purpura.

View Media Gallery

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

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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-Barré 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 patient’s 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.

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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-Schönlein 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.

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Typical rash distribution of Henoch-Schönlein purpura.

View Media Gallery

A 9-year-old boy with Henoch-Schönlein purpura. Note confluence of purpura around the ankles.
Image courtesy of Pamela L Dyne, MD.

View Media Gallery

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

View Media Gallery

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Hemorrhagic macules, papules, and patches on the ankle and foot of a child with Henoch-Schönlein
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-Schönlein purpura demonstrating increased extravasation with ecchymoses

on dorsal foot and ankle.

View Media Gallery

Hives, angioedema, and target lesions can also occur. Vesicular eruptions and swelling and
tenderness of an entire limb have been noted. Erythema multiforme–like 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

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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.

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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

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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]

Differential Diagnoses

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Media Gallery

Purpuric papules and plaques of the lower extremity characteristic of Henoch-

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Henoch-Schonlein Purpura Clinical Presentation: History, Physica... http://emedicine.medscape.com/article/984105-clinical

Schönlein purpura.
Hemorrhagic macules, papules, and patches on the ankle and foot of a child with
Henoch-Schönlein purpura.
Typical rash distribution of Henoch-Schönlein purpura.
Characteristic rash of Henoch-Schönlein purpura.
Older lesions of Henoch-Schönlein purpura demonstrating increased extravasation
with ecchymoses on dorsal foot and ankle.
A 9-year-old boy with Henoch-Schönlein purpura. Note confluence of purpura around
the ankles. Image courtesy of Pamela L Dyne, MD.
A 7-year-old girl with Henoch-Schönlein purpura. Image courtesy of Pamela L Dyne,
MD.

of 7

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Contributor Information and Disclosures

Author

Noah S Scheinfeld, JD, MD, FAAD Assistant Clinical Professor, Department of

Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St
Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear
Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant
Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private
Practice

Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American
Academy of Dermatology

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for:

Abbvie<br/>Received income in an amount equal to or greater than $250 from:
Optigenex<br/>Received salary from Optigenex for employment.

Coauthor(s)

Elena L Jones, MD Clinical Assistant Professor of Dermatology, Columbia University

College of Physicians and Surgeons; Clinic Chief, Department of Dermatology, St Luke's-
Roosevelt Hospital Center

Disclosure: Nothing to disclose.

Chief Editor

Craig B Langman, MD The Isaac A Abt, MD, Professor of Kidney Diseases, Northwestern

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University, The Feinberg School of Medicine; Division Head of Kidney Diseases, The Ann
and Robert H Lurie Children's Hospital of Chicago

Craig B Langman, MD is a member of the following medical societies: American Academy of

Pediatrics, American Society of Nephrology, International Society of Nephrology

Disclosure: Received income in an amount equal to or greater than $250 from: Alexion
Pharmaceuticals; Raptor Pharmaceuticals (now Horizon Pharmaceuticals); ; Dicerna, QLT
Pharmaceuticals.

Acknowledgements

Jeffrey L Arnold, MD, FACEP Chairman, Department of Emergency Medicine, Santa Clara
Valley Medical Center

Jeffrey L Arnold, MD, FACEP is a member of the following medical societies: American
Academy of Emergency Medicine and American College of Physicians

Disclosure: Nothing to disclose.

Philip Bossart, MD Professor, Department of Surgery, Division of Emergency Medicine,

University of Utah Hospital, University of Utah School of Medicine

Philip Bossart, MD is a member of the following medical societies: American College of

Emergency Physicians

Disclosure: Nothing to disclose.

Steven C Dronen, MD, FAAEM Chair, Department of Emergency Medicine, LeConte

Medical Center

Steven C Dronen, MD, FAAEM is a member of the following medical societies: American
Academy of Emergency Medicine and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Pamela L Dyne, MD Professor of Clinical Medicine/Emergency Medicine, University of

California, Los Angeles, David Geffen School of Medicine; Attending Physician, Department
of Emergency Medicine, Olive View-UCLA Medical Center

Pamela L Dyne, MD is a member of the following medical societies: American Academy of

Emergency Medicine, American College of Emergency Physicians, and Society for
Academic Emergency Medicine

Disclosure: Nothing to disclose.

Gina M Forte Medical Assistant, The Skin Cancer Surgery Center

Disclosure: Nothing to disclose.

Edmond A Hooker II, MD, DrPH, FAAEM Associate Professor, Department of Health
Services Administration, Xavier University, Cincinnati, Ohio; Assistant Professor, Department

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of Emergency Medicine, University of Cincinnati College of Medicine

Edmond A Hooker II, MD, DrPH, FAAEM is a member of the following medical societies:
American Academy of Emergency Medicine, American Public Health Association, Society for
Academic Emergency Medicine, and Southern Medical Association

Disclosure: Nothing to disclose.

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency

Program Director, Department of Dermatology, University of Pennsylvania School of
Medicine

William D James, MD is a member of the following medical societies: American Academy of

Dermatology and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Heather Kesler DeVore, MD Assistant Professor, Clinical Attending Physician, Department

of Emergency Medicine, Georgetown University Hospital and Washington Hospital Center

Heather Kesler DeVore, MD is a member of the following medical societies: Emergency

Medicine Residents Association and Society for Academic Emergency Medicine

Disclosure: Nothing to disclose.

Andrew D Montemarano, DO Consulting Staff, The Skin Cancer Surgery Center

Andrew D Montemarano, DO is a member of the following medical societies: American

Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous
Oncology, American Society for Dermatologic Surgery, and MedChi

Disclosure: Nothing to disclose.

Richard Neiberger, MD, PhD Director of Pediatric Renal Stone Disease Clinic, Associate
Professor, Department of Pediatrics, Division of Nephrology, University of Florida College of
Medicine and Shands Hospital

Richard Neiberger, MD, PhD is a member of the following medical societies: American
Academy of Pediatrics, American Federation for Medical Research, American Medical
Association, American Society of Nephrology, American Society of Pediatric Nephrology,
Christian Medical & Dental Society, Florida Medical Association, International Society for
Peritoneal Dialysis, International Society of Nephrology, National Kidney Foundation, New
York Academy of Sciences, Shock Society, Sigma Xi, Southern Medical Association,
Southern Society for Pediatric Research, and Southwest Pediatric Nephrology Study Group

Disclosure: Nothing to disclose.

Julia R Nunley, MD Professor, Program Director, Dermatology Residency, Department of

Dermatology, Virginia Commonwealth University Medical Center

Julia R Nunley, MD is a member of the following medical societies: American Academy of

Dermatology, American College of Physicians, American Society of Nephrology, International

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Society of Nephrology, Medical Dermatology Society, Medical Society of Virginia, National

Kidney Foundation, Phi Beta Kappa, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Stacy Sawtelle, MD Clinical Instructor, Department of Emergency Medicine, University of

California, San Francisco, School of Medicine

Disclosure: Nothing to disclose.

Debra Slapper, MD Consulting Staff, Department of Emergency Medicine, St Anthony's

Hospital

Debra Slapper, MD is a member of the following medical societies: American Academy of

Emergency Medicine

Disclosure: Nothing to disclose.

Adrian Spitzer, MD Clinical Professor Emeritus, Department of Pediatrics, Albert Einstein

College of Medicine

Adrian Spitzer, MD is a member of the following medical societies: American Academy of

Pediatrics, American Federation for Medical Research, American Pediatric Society,
American Society of Nephrology, American Society of Pediatric Nephrology, International
Society of Nephrology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska

Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Michael J Wells, MD Associate Professor, Department of Dermatology, Texas Tech

University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha,
American Academy of Dermatology, American Medical Association, and Texas Medical
Association

Disclosure: Nothing to disclose.

Robert J Willard, MD Dermatologist and Mohs Surgeon, Private Practice, Dermatology and
Mohs Surgery Center, PC

Robert J Willard, MD is a member of the following medical societies: American Academy of

Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology,
and American Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical

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Henoch-Schonlein Purpura Clinical Presentation: History, Physica... http://emedicine.medscape.com/article/984105-clinical

Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Wayne Wolfram, MD, MPH Professor, Department of Emergency Medicine, Mercy St

Vincent Medical Center; Chairman, Pediatric Institutional Review Board, Mercy St Vincent
Medical Center, Toledo, Ohio

Wayne Wolfram, MD, MPH, is a member of the following medical societies: American
Academy of Emergency Medicine, American Academy of Pediatrics, and Society for
Academic Emergency Medicine

Disclosure: Nothing to disclose.

David Timothy Woodley, MD Professor and Chair, Department of Dermatology, Keck

School of Medicine of the University of Southern California

David Timothy Woodley, MD is a member of the following medical societies: American

Academy of Dermatology, American Association for the Advancement of Science, American
College of Emergency Physicians, American College of Physicians, American Federation for
Medical Research, American Society for Clinical Investigation, New York Academy of
Medicine, Society for Investigative Dermatology, and Southern Medical Association

Disclosure: Lotus Tissue Repair Ownership interest owner and advisor

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