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Henoch-Schonlein Purpura in Emergency Medicine Background

Henoch-Schnlein purpura (HSP) is a small-vessel vasculitis characterized by purpura, arthritis, abdominal pain, and hematuria.[1, 2] 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 Henoch-Schnlein purpura. However, the illness is named after the 2 German physicians who further characterized this vasculitis. In 1837, Johan Schnlein 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
Henoch-Schnlein purpura (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.[2, 3, 4]

Epidemiology
Frequency
United States Approximately 14 cases of Henoch-Schnlein purpura occur per 100,000 school-aged children. Henoch-Schnlein purpura also occurs in adults, although less commonly than in children.[1]

Mortality/Morbidity
In general, Henoch-Schnlein purpura (HSP) is a benign self-limited disorder. y Less than 5% of cases cause chronic symptoms. y Less than 1% of cases progress to end-stage renal failure.

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

Age
Approximately 75% of patients affected by Henoch-Schnlein purpura are aged 2-11 years. In some series, as many as 27% of the patients are adults.[4]

History

The most common symptoms of Henoch-Schnlein purpura include the following: y Rash (95-100%), especially involving the legs, may not be present on initial presentation y Subcutaneous edema (20-50%) y Abdominal pain and vomiting (85%) y Joint pain (60-80%), especially involving the knees and ankles y Scrotal edema (2-35%) y Bloody stools

Physical
Physical examination findings of Henoch-Schnlein purpura may include the following: y Palpable purpura, particularly on the buttocks and legs (shown in the images below)

Typical rash

distribution

of

Henoch-Schnlein

purpura.

y y y y y

Characteristic rash of Henoch-Schnlein purpura. 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
y y y y

The etiology of Henoch-Schnlein purpura 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 Henoch-Schnlein purpura. Antistreptolysin O titers are raised in 20-50% of patients.

Differentials
y y y y y y

Disseminated Intravascular Coagulation Endocarditis Pancreatitis Pediatrics, Meningitis and Encephalitis Testicular Torsion Thrombocytopenic Purpura

Laboratory Studies
y y

y y

y y y y

Diagnosis of Henoch-Schnlein purpura 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 Henoch-Schnlein purpura. 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 Henoch-Schnlein purpura (HSP) or from dehydration.

Imaging Studies
y

Abdominal ultrasonography may be better than barium enema to diagnose intussusception, since Henoch-Schnlein purpura (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 Henoch-Schnlein purpura, in contrast to the decreased blood flow seen in testicular torsion.

Emergency Department Care

Treatment of Henoch-Schnlein 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 Summary
Very limited data are available on the treatment of Henoch-Schnlein purpura. Fortunately, most patients recover quickly in several weeks without treatment.[5] 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 subcutaneous edema and nephritis, but few prospective placebo-controlled studies have demonstrated their effectiveness. Randomized controlled trials do not support the use of steroids to prevent or treat renal disease.[6] [7] Some authors recommend steroids and others do not. Good, large, prospective studies regarding the treatment of Henoch-Schnlein purpura (HSP) are lacking.[8, 9, 10] 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.[11] 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. One study of 12 patients with severe Henoch-Schnlein purpura (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.[12]

Nonsteroidal anti-inflammatory drugs (NSAIDs)

Class Summary
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.

Ibuprofen (Ibuprin, Advil, Motrin)

DOC for treatment of mild to moderately severe pain, if no contraindications. Inhibits inflammatory reactions and pain, probably by decreasing activity of enzyme cyclooxygenase, which results in inhibition of prostaglandin synthesis.

Flurbiprofen (Ansaid)
Has analgesic, antipyretic, and anti-inflammatory effects; cyclooxygenase, causing inhibition of prostaglandin biosynthesis. may inhibit enzyme

Ketoprofen (Oruvail, Orudis, Actron)

Used for relief of mild to moderately severe pain and inflammation.

Naproxen (Anaprox, Naprelan, Naprosyn)

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.

Corticosteroids
Class Summary
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.

Prednisone (Deltasone, Sterapred, Orasone)

Useful in treatment of inflammatory and autoimmune reactions. By reversing increased capillary permeability and suppressing PMN activity, may decrease inflammation.

Further Inpatient Care

y

Patients with Henoch-Schnlein purpura who have severe abdominal pain, significant gastrointestinal bleeding, or marked renal insufficiency may require hospitalization.

Prognosis
y y y y y

Henoch-Schnlein purpura (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 Henoch-Schnlein purpura. Abdominal pain resolves spontaneously within 72 hours in most patients.

Patient Education
y

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