AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

INTRODUCTION

Section 2 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Background

Henoch-Schönlein purpura (HSP) is a small-vessel vasculitis characterized by purpura, arthritis, abdominal pain, and hematuria. In his 1801 book, On Cutaneous Diseases, Heberden described a 5-year-old boy with "bloody points" over the skin of his legs, abdominal pain, bloody stools and urine, and painful subcutaneous edema. This may be the first published case of HSP. However, the illness is named after the 2 German physicians who further characterized this vasculitis.

In 1837, Johan Schönlein described the association of nonthrombocytopenic purpura and joint pain, which he called purpura rheumatica. Later, his student, Eduard Henoch, noted the gastrointestinal and renal involvement in this disease.

Pathophysiology

HSP is a small-vessel vasculitis characterized by immunoglobulin A (IgA), C3, and immune complex deposition in arterioles, capillaries, and venules. HSP and IgA nephropathy are related disorders. Both illnesses have elevated serum IgA levels and identical findings on renal biopsy; however, IgA nephropathy almost exclusively involves young adults and predominantly affects the kidneys only. HSP affects mostly children and involves the skin and connective tissues, gastrointestinal tract, joints, and scrotum as well as the kidneys.

Frequency

United States

Approximately 14 cases occur per 100,000 school-aged children. HSP also occurs in adults, although less commonly than in children.

Mortality/Morbidity

In general, HSP is a benign self-limited disorder.

• Less than 5% of cases cause chronic symptoms.
• Less than 1% of cases progress to end-stage renal failure.

Sex

The male-to-female ratio is about 2:1.

Age

Seventy-five percent of patients affected are aged 2-11 years. In some series, as many as 27% of the patients are adults.

CLINICAL

Section 3 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

History

The most common symptoms of Henoch-Schönlein purpura include the following:

• Rash (95-100%), especially involving the legs, may not be present on initial presentation
• Subcutaneous edema (20-50%)
• Abdominal pain and vomiting (85%)
• Joint pain (60-80%), especially involving the knees and ankles
• Scrotal edema (2-35%)
• Bloody stools

Physical

• Palpable purpura, particularly on the buttocks and legs (see Media files 1-2)
• Edema of the hands, feet, scalp, and ears
• Arthritis, most commonly involving the knees and ankles
• Abdominal tenderness
• Gastrointestinal bleeding
• Acute scrotal edema that may mimic testicular torsion

Causes

• The etiology of HSP is unknown.
• About 50% of patients have a preceding upper respiratory illness (URI).
• Multiple infectious agents as well as drugs, foods, and insect bites may trigger HSP.
• Antistreptolysin O titers are raised in 20-50% of patients.

DIFFERENTIALS

Section 4 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Other Problems to be Considered

Essential mixed cryoglobulinemia
Waldenström macroglobulinemia
Systemic lupus erythematosus
Rickettsial diseases
Bowel infarction/perforation

WORKUP

Section 5 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Lab Studies

• Diagnosis is clinical and 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, CBC with platelet count and differential, BUN level, creatinine level, prothrombin time (PT), activated partial thromboplastin time (aPTT), and lipase level.
• Urinalysis: Hematuria and/or proteinuria are present in 10-20% of patients.
• Platelet count and coagulation studies: Platelet count is usually in the reference range but may be elevated; the platelet count should not be low in HSP. A normal platelet count rules out idiopathic thrombocytopenic purpura (ITP). A normal platelet count and normal coagulation studies (ie, PT, aPTT, fibrin split products) rule out thrombotic thrombocytopenic purpura (TTP).
• Lipase: A normal lipase level makes acute pancreatitis very unlikely.
• CBC and differential: WBC count may be in the reference range or elevated. Eosinophilia is sometimes present.
• Erythrocyte sedimentation rate: Sedimentation rate may be in the reference range or elevated.
• BUN and creatinine levels: These may be elevated from renal involvement of HSP or from dehydration.

Imaging Studies

• Abdominal ultrasonography may be better than barium enema to diagnose intussusception, since HSP-related intussusception is more often ileoileal instead of ileocolic as is typical in idiopathic intussusception.
• 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.

TREATMENT

Section 6 of 11  

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• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Emergency Department Care

Treatment of Henoch-Schönlein purpura is mostly supportive and includes ensuring adequate hydration and monitoring for abdominal and renal complications.

Consultations

Consultation is recommended for patients with renal involvement prior to discharge from the ED and for all patients who appear acutely ill.

MEDICATION

Section 7 of 11  

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• Follow-up
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Very limited data are available on the treatment of Henoch-Schönlein purpura. Fortunately, most patients recover quickly in several weeks without treatment. 

Nonsteroidal anti-inflammatory drugs (NSAIDs) may help joint pain and do not seem to worsen the purpura. However, NSAIDs should be used cautiously in patients with renal insufficiency.

Clinicians often use corticosteroids to treat abdominal pain, subcutaneous edema, and nephritis, but few prospective placebo-controlled studies have demonstrated their effectiveness.

Prednisone in a dose of 1 mg/kg/d for 2 weeks and then tapered over 2 more weeks has been shown to improve gastrointestinal and joint symptoms. Although this regimen did not decrease the incidence of renal disease, it did lessen the severity of nephritis in some patients. 

Other treatment regimens have included IV or oral steroids with or without any of the following: azathioprine, cyclophosphamide, cyclosporine, dipyridamole, plasmapheresis, high-dose IV immunoglobulin G (IVIg), danazol, or fish oil. A recent study of 12 patients with severe HSP nephritis indicated that patients did well with a treatment of methylprednisolone at 30 mg/kg/d for 3 days followed by oral corticosteroids at 2 mg/kg/d for 2 months, cyclophosphamide at 2 mg/kg/d for 2 months, and dipyridamole at 5 mg/kg/d for 6 months.

Drug Category: Nonsteroidal anti-inflammatory drugs (NSAIDs)

These agents are most commonly used for the relief of mild to moderately severe pain. Although the effects of NSAIDs in the treatment of pain tend to be patient specific, ibuprofen usually is the DOC for initial therapy. Other options include flurbiprofen, ketoprofen, and naproxen.

Drug Name	Flurbiprofen (Ansaid)
Description	Has analgesic, antipyretic, and anti-inflammatory effects; may inhibit enzyme cyclooxygenase, causing inhibition of prostaglandin biosynthesis.
Adult Dose	200-300 mg/d PO divided bid/qid
Pediatric Dose	Not established
Contraindications	Documented hypersensitivity
Interactions	Aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT in patients taking anticoagulants (monitor PT closely and instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; may increase phenytoin levels
Pregnancy	B - Usually safe but benefits must outweigh the risks. D - Unsafe in pregnancy
Precautions	Acute renal insufficiency, interstitial nephritis, hyperkalemia, hyponatremia, and renal papillary necrosis may occur; patients with preexisting renal disease or compromised renal perfusion risk acute renal failure; leukopenia occurs rarely, is transient, and usually returns to normal during therapy; persistent leukopenia, granulocytopenia, or thrombocytopenia warrants further evaluation and may require discontinuation of drug

Drug Name	Ketoprofen (Oruvail, Orudis, Actron)
Description	Used for relief of mild to moderately severe pain and inflammation.
Adult Dose	25-50 mg PO q6-8h prn; not to exceed 300 mg/d
Pediatric Dose	<3 months: Not established 3 months to 12 years: 0.1-1 mg/kg PO q6-8h >12 years: Administer as in adults
Contraindications	Documented hypersensitivity
Interactions	Aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity—it increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT in patients taking anticoagulants (monitor PT closely and instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; may increase phenytoin levels
Pregnancy	B - Usually safe but benefits must outweigh the risks. D - Unsafe in pregnancy
Precautions	Administer small dosages initially to patients with small body size, to those who are elderly, and to those with renal or liver disease; doses higher than 75 mg do not increase therapeutic effects; administer high doses with caution and closely observe patient for response; avoid in GI disease, cardiovascular disease, and renal or hepatic impairment; acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; caution in patients who have coagulation defects or are receiving anticoagulant therapy

Drug Name	Naproxen (Anaprox, Naprelan, Naprosyn)
Description	Used for relief of mild to moderately severe pain. Inhibits inflammatory reactions and pain by decreasing activity of enzyme cyclooxygenase, which results in decrease of prostaglandin synthesis.
Adult Dose	250-500 mg PO bid; may increase to 1.5 g/d for limited periods
Pediatric Dose	2.5 mg/kg/dose PO; not to exceed 10 mg/kg/d
Contraindications	Documented hypersensitivity; peptic ulcer disease; recent GI bleeding or perforation; renal insufficiency; high risk of bleeding
Interactions	Aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity; may decrease effects of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT in patients taking anticoagulants (monitor PT closely and instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; may increase phenytoin levels
Pregnancy	B - Usually safe but benefits must outweigh the risks. D - Unsafe in pregnancy
Precautions	Acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; low WBC counts occur rarely and usually return to normal during ongoing therapy; discontinuation of therapy may be necessary if leukopenia, granulocytopenia, or thrombocytopenia persists; exercise caution in patients who have coagulation defects or are receiving anticoagulant therapy

Drug Category: Corticosteroids

These agents have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, they modify the body's immune response to diverse stimuli.

FOLLOW-UP

Section 8 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Further Inpatient Care

• Patients with severe abdominal pain, significant gastrointestinal bleeding, or marked renal insufficiency may require hospitalization.

Prognosis

• HSP is generally a benign disease with an excellent prognosis.
• More than 80% of patients have a single isolated episode lasting a few weeks.
• Approximately 10-20% of patients have recurrences.
• Fewer than 5% of patients develop chronic HSP.
• Abdominal pain resolves spontaneously within 72 hours in most patients.

Patient Education

• For excellent patient education resources, visit eMedicine's Kidneys and Urinary System Center. Also, see eMedicine's patient education article Blood in the Urine.

MISCELLANEOUS

Section 9 of 11  

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• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Medical/Legal Pitfalls

• Failure to differentiate scrotal edema, which may be a common finding, from testicular torsion
• Failure to perform the required evaluation in patients with severe abdominal pain to rule out intussusception, bowel perforation, bowel ischemia, or pancreatitis

MULTIMEDIA

Section 10 of 11  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

Media file 1:  Typical rash distribution of Henoch-Schönlein purpura.
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Media type:  Photo

Media file 2:  Characteristic rash of Henoch-Schönlein purpura.
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Media type:  Photo

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• Multimedia
• References

• Ronkainen J, Koskimies O, Ala-Houhala M, et al. Early prednisone therapy in Henoch-Schonlein purpura: a randomized, double-blind, placebo-controlled trial. J Pediatr. Aug 2006;149(2):241-7. [Medline].
• Blanco R, Martínez-Taboada VM, Rodriguez-Valverde V, García-Fuentes M, Gonzalez-Gay MA. Henoch-Schonlein purpura in adulthood and childhood: two different expressions of the same syndrome. Arthritis Rheum. May 1997;40(5):859-64. [Medline].
• Gedalia A. Henoch-Schönlein purpura. Curr Rheumatol Rep. Jun 2004;6(3):195-202. [Medline].
• O'Brien WM, O'Connor KP, Horan JJ, Eggli DF, Gibbons MD. Acute scrotal swelling in Henoch-Schonlein syndrome: evaluation with testicular scanning. Urology. Apr 1993;41(4):366-8. [Medline].
• Pillebout E, Thervet E, Hill G, Alberti C, Vanhille P, Nochy D. Henoch-Schonlein Purpura in adults: outcome and prognostic factors. J Am Soc Nephrol. May 2002;13(5):1271-8. [Medline].
• Szer IS. Henoch-Schonlein purpura: when and how to treat. J Rheumatol. Sep 1996;23(9):1661-5. [Medline].
• Szer IS. Henoch-Schonlein purpura. Curr Opin Rheumatol. Jan 1994;6(1):25-31. [Medline].
• Saulsbury FT. Clinical update: Henoch-Schönlein purpura. Lancet. Mar 24 2007;369(9566):976-8. [Medline].

Article Last Updated: Jul 18, 2007