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Pediatrics, Henoch-Schonlein Purpura
Updated: Aug 13, 2009
Introduction
Background
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.
A 9-year-old boy with Henoch-Schönlein purpura. Note confluence of purpura around the ankles. Courtesy of Pamela L Dyne, MD.
A 7-year-old girl with Henoch-Schönlein purpura. Courtesy of Pamela L Dyne, MD.
Pathophysiology
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.
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.
Frequency
United States
The rate is 14 cases per 100,000 population.
Mortality/Morbidity
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.
Clinical
History
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.
Physical
- Skin
- 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.
Causes
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
- Drugs associated with Henoch-Schönlein purpura
- Penicillin
- Ampicillin
- Erythromycin
- Quinidine
- Quinine
- Vaccines associated with Henoch-Schönlein purpura
- Typhoid and paratyphoid A and B
- Measles
- Yellow fever
- Cholera
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References
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Further Reading
Keywords
Henoch-Schönlein purpura, Henoch-Schonlein purpura, rheumatica purpura, leukocytoclastic vasculitis, allergic vasculitis, HSP, end-stage renal disease, ESRD, intussusception, bowel infarction, bowel perforation, hydrops of the gallbladder, pancreatitis, GI bleeding, chronic renal disease, arthritis, proteinuria, abdominal pain, erythematous macules, urticarial papules, pruritic papules, plaques, measles, yellow fever, cholera, treatment, diagnosis
