Henoch-Schönlein purpura (HSP) is an inflammatory disorder characterized by a generalized vasculitis involving the small vessels of the skin, GI tract, kidneys, joints, and, rarely, the lungs and CNS. It is the most common vasculitis in children.
The syndrome takes its name from 2 German physicians. In 1837, Johan Schönlein 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-Schönlein purpura has also been referred to as rheumatica purpura, leukocytoclastic vasculitis, and allergic vasculitis. See the images below.
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A 9-year-old boy with Henoch-Schönlein purpura. Note confluence of purpura around the ankles. Courtesy of Pamela L Dyne, MD.
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A 7-year-old girl with Henoch-Schönlein purpura. Courtesy of Pamela L Dyne, MD.
The etiology of Henoch-Schönlein purpura is unclear. It is thought to be multifactorial with genetic, environmental, and antigenic components. More than 75% of patients report antecedent upper-respiratory, pharyngeal, or GI infections. Multiple bacterial and viral infectious agents have been associated with the development of Henoch-Schönlein purpura, and cases of Henoch-Schönlein purpura also have been reported after drug ingestions and vaccinations.[1]
Henoch-Schönlein purpura is thought to be an immunoglobulin A (IgA)–mediated autoimmune phenomenon. An unknown antigenic stimulant has been postulated to cause a rise in IgA. The antigen-antibody complexes deposit locally throughout the body and activate pathways leading to necrotizing vasculitis.
Genetic research may reveal the potential role of cytokines, endothelia and nitric oxide metabolism in Henoch-Schönlein purpura.
Henoch-Schönlein purpura can involve nearly any organ system. Hallmarks of Henoch-Schönlein purpura include a characteristic rash, migratory polyarthritis, renal involvement, and GI involvement. The clinical manifestations of Henoch-Schönlein purpura are the result of antigen-antibody complexes depositing throughout the body, which cause migratory arthralgias, abdominal cramping, the petechial and/or vasculitic rash, and hematuria.
Henoch-Schönlein purpura generally resolves without permanent complications. However, serious GI and renal complications may occur. GI complications include intussusception (usually ileoileal), bowel infarction, bowel perforation, hydrops of the gallbladder, pancreatitis, or massive GI bleeding.
Approximately 20% of patients have renal manifestations, and 5% develop end-stage renal disease (ESRD). Patients with only hematuria do not develop ESRD. About 15% of patients with hematuria and proteinuria develop ESRD. Approximately 50% of patients with nephritic or nephrotic syndrome develop ESRD. The long-term morbidity is predominantly attributed to renal involvement.
Sex
In children, the male-to-female ratio is 2:1. In adults, the male-to-female ratio is approximately 1:1.
Age
Henoch-Schönlein purpura primarily affects children. Adults are rarely affected. Approximately 75% of cases occur in children aged 2-11 years. The median age is 5 years. Older age at disease onset is associated with development of chronic renal disease.[2]
The following may be noted in the history of patients with Henoch-Schönlein purpura (HSP):
The prodrome is associated with the following:
Headache
Anorexia
Fever
After the prodrome, a rash, abdominal pain, peripheral edema, vomiting and/or arthritis develop.
The rash appears in 100% of patients and is the presenting feature in 50%.
The distribution usually depends on parts of the body, including the lower trunk, lower extremities, buttocks and perineum.
The rash typically appears in crops with new crops appearing in waves.
Eruptions usually last an average of 3 weeks.
As many as 85% of patients will have GI symptoms, including abdominal pain, nausea, and vomiting.
The most common symptom is colicky abdominal pain.
Joint involvement is present in 75% of reported patients with Henoch-Schönlein purpura and the presenting sign in approximately 25%.
The large joints (eg, knees and ankles) are most commonly involved, with pain and edema being the only symptoms. The arthritis resolves completely over several days without permanent articular damage.
Renal involvement is present in 30-50% of patients and may persist as long as 6 months after the onset of the rash.
Renal involvement manifests in a range from mild hematuria or proteinuria to oliguria and renal failure.
Permanent renal impairment is seen in 20% of patients who have nephrotic or nephritic syndrome; however, this turns out to be less than 0.1 % of all patients diagnosed with Henoch-Schönlein purpura.
Lesions consist of erythematous macules, urticarial papules, pruritic papules, and plaques. Skin lesions tend to appear in crops in the dependent portions of the body (eg, lower extremities, lower abdomen, buttocks).
Children younger than 2 years also may have involvement of the upper extremity, head, and trunk.
The rash typically appears as red macules and papules, which later become purple and then rust-colored.
Various stages of eruption are usually present simultaneously. The lesions may blanch initially, but they progress to palpable purpura as they mature.
Abdomen
Heme-positive stool is the primary finding on GI examination.
Findings on abdominal examination are generally unremarkable.
On occasion, the abdomen is tender.
Signs of an acute abdomen are rarely present.
Joints
The knees, ankles, and (less commonly) wrists are involved.
Tenderness and edema are periarticular. Warmth, erythema, and effusions are not typically associated with Henoch-Schönlein purpura.
Other
Case reports describe patients with Henoch-Schönlein purpura presenting with protein-losing enteropathy without liver or kidney dysfunction.
The rash may appear late in the course, simplifying the diagnosis.
The current understanding of the etiology of Henoch-Schönlein purpura suggests the involvement of toxins, viruses, idiopathic causes, and drugs. No single etiology has been clearly identified; however, most cases are preceded by a recent upper airway infection.
Infectious agents associated with Henoch-Schönlein purpura
Electrolyte values in patients with Henoch-Schönlein purpura (HSP) are generally in the reference range, but excessive vomiting can affect the values.
BUN and creatinine levels may be increased in the presence of renal involvement.
Amylase and lipase levels may be elevated in patients with pancreatitis.
A CBC count usually reveals a leukocytosis with a left shift, possibly eosinophilia, and a normal or increased platelet count. Hemoglobin and/or hematocrit values may be normal or decreased secondary to bleeding.
Urinalysis usually shows hematuria, proteinuria, and occasional red cell casts.
Additional laboratory tests that can be helpful in narrowing the differential diagnosis include the following:
Assessment of antistreptolysin-O (ASO) titer
Monospot test
Antinuclear antibody (ANA) test
Rheumatoid factor (RF) test
Determination of C3/C4 levels
Measurement of the prothrombin time (PT)
Measurement of the activated partial thromboplastin time (aPTT)
ED treatment of Henoch-Schönlein purpura (HSP) is supportive, with frequent monitoring of vital signs. 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.
Most patients with self-limited cases can be safely discharged home with close follow-up by their primary physician. Whether or not to admit the patient to the hospital depends on the practice of the admitting pediatrician and his or her 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.
One study examined the prevention and treatment of renal disease in patients with Henoch-Schönlein purpura.[3] Meta-analyses of 4 trials 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, compared with placebo or supportive treatment. 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. However, data from randomized trials for any intervention used to improve renal outcome in children with Henoch-Schönlein purpura are sparse.
All unnecessary drugs should be discontinued if the etiology is suspected to be drug related.
Patients with renal involvement require close attention in regard to their fluid balance, electrolyte status, and use of antihypertensives (if indicated).
Use of immunosuppressive and cytotoxic drugs is gaining favor based on research and case studies.
Dapsone has been used to treat associated purpura and arthralgias.
Factor VIII concentrate has been used to relieve abdominal pain when corticosteroids are contraindicated.
Plasmapheresis is currently under investigation.
Kidney transplantation may be indicated in patients with severe renal disease that is resistant to medical therapy.
Surgery may be undertaken to treat severe bowel ischemia.
Treatment of Henoch-Schönlein purpura (HSP) is largely supportive. Analgesia with nonsteroidal anti-inflammatory drugs (NSAIDs) or acetaminophen may reduce joint and soft tissue discomfort. The evidence does not clearly demonstrate that corticosteroid administration prevents the development of nephritis in patients with Henoch-Schönlein purpura, although its use in the treatment of intestinal and neurologic complications is gaining acceptance. If used, prednisone 1-2 mg/kg/d PO for 7 days is recommended. Antihypertensives may be indicated with renal involvement; for more information, see the pediatric topic Hypertension.
Other drugs are currently under investigation (see Emergency Department Care). For more information on long-term medication management, see the pediatric general medicine topic Henoch-Schönlein Purpura.
Clinical Context:
Effective for treating fever or mild-to-moderate pain. Inhibits inflammatory reactions and pain by decreasing prostaglandin synthesis.
Clinical Context:
Inhibits action of endogenous pyrogens on heat-regulating centers; reduces fever by a direct action on the hypothalamic heat-regulating centers, which, in turn, increase the dissipation of body heat via sweating and vasodilation. Effective for treating fever and relieving mild-to-moderate pain.
Clinical Context:
Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and also suppresses lymphocyte and antibody production.
Patients with Henoch-Schönlein purpura (HSP) have the potential for severe complications, which may occur precipitously (eg, acute abdomen, acute scrotum, renal failure).
Whether or not to admit the patient to the hospital for observation and monitoring depends on the practice of the admitting pediatrician and his or her preference.
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 are normal.
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
Disclosure: Nothing to disclose.
Coauthor(s)
Heather Kesler DeVore, MD, Assistant Professor, Clinical Attending Physician, Department of Emergency Medicine, Georgetown University Hospital and Washington Hospital Center
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.
Specialty Editors
Debra Slapper, MD, Consulting Staff, Department of Emergency Medicine, St Anthony's Hospital
Disclosure: Nothing to disclose.
Mary L Windle, PharmD, Adjunct Associate Professor, University of Nebraska Medical 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
Disclosure: Nothing to disclose.
John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
Disclosure: Nothing to disclose.
Chief Editor
Richard G Bachur, MD, Associate Professor of Pediatrics, Harvard Medical School; Associate Chief and Fellowship Director, Attending Physician, Division of Emergency Medicine, Children's Hospital of Boston
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