Henoch-Schönlein Purpura

Definition

  • IgA immune-complex mediated vasculitis of small vessels
  • characterized by leukocytoclastic vasculitis and IgA deposition in the small vessels in the skin, joints, gastrointestinal tract, and kidney

Clinical Manifestations

  • purpuric rash, palpable
    • starting as pink macules or wheals and developing into petechiae, raised purpura, or larger ecchymoses
    • occur in lower extremities, buttocks
    • typically lasting 3-10 days, and may recur up to 4 mo after initial presentation
    • subcutaneous edema localized to the dorsa of hands and feet, periorbital area, lips, scrotum, or scalp is also common
  • arthritis, arthralgia
    • knees, ankles, elbows, and wrists are commonly affected
  • abdominal pain, vomiting, diarrhea, paralytic ileus, melena
    • usually related to mesenteric vasculitis
  • nephritis
    • characterized by proteinuria, hematuria, and hypertension, frank nephritis, nephrotic syndrome, and acute or chronic renal failure
  • neurological manifestation
    • caused by hypertension or central nervous system (CNS) vasculitis,
    • e.g. intracerebral hemorrhage, seizures, headaches, depressed level of consciousness, cranial or peripheral neuropathies, and behavior changes

Differential Diagnosis

  • other small vessel vasculitides

  • infections

  • acute poststreptococcal glomerulonephritis

  • hemolytic-uremic syndrome

  • coagulopathies

  • papular-purpuric glove and sock syndrome

  • SLE

  • other vasculitides (urticarial, hypersensitivity)

  • thrombocytopenia

  • infantile acute hemorrhagic edema (AHE)

    • isolated cutaneous leukocytoclastic vasculitis that affects infants <2 yr of age
    • resembles HSP clinically
    • manifests as fever; tender edema of the face, scrotum, hands, and feet; and ecchymosis (usually larger than the purpura of HSP) on the face and extremities, trunk is spared
  • Reference

    • Nelson page 2131/6873