UMEM Educational Pearls

Category: Pediatrics

Title: Henoch-Schonlein Purpura

Posted: 8/3/2012 by Lauren Rice, MD (Updated: 4/23/2024)
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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