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Henoch-Schonlein Purpura (aka. Anaphylactoid purpura) is a small vessel vasculitis.
Background:
- most commonly diagnosed vasculitide in childhood
- age range 3-15 years, mean age 4yo, mostly <7yo (75% cases)
- more cases in Winter and Spring months
- boys more commonly than girls (2:1)
- IgA-mediated leukoclastic vasculitis
Clinical Features:
- Rash: progresses to petechiae, purpura; occurs on lower extremities and buttocks in dependent areas
- Joints: arthritis/arthralgia mainly of large joints (knees, ankles)
- GI: colicky abdominal pain, may occur with melena (33%) or less likely, hematemesis; ultrasound for intussusception (2-14%)
- Renal: microscopic hematuria with/without proteinuria; usually transient but may lead to progressive renal disease in patients with more severe, persistent symptoms
- Orchitis and/or angioedema may also occur
Etiology:
- unknown
- preceding URI (50%)
- associated with bacteria (Strep pyogenes, Legionella, Mycoplasma), viruses (EBV, CMV, parvovirus), drugs (penicillin, cephalosporins), and insect bites
Diagnosis:
- clinical features
- lab studies that are helpful but nonspecific: high WBC, high ESR, high IgA, normal platelet and coagulation studies
Treatment:
- supportive care, may last up to 4 weeks
- steroids may be helpful but evidence has not shown true benefit
- recurrence happens in 40% of cases