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Immunoglobulin A Vasculitis

Differential Diagnosis

  • Acute meningococcemia
  • Erythema elevatum diutinum
  • Immunoglobulin A vasculitis
  • Systemic lupus erythematosus

Diagnosis

The correct diagnosis in this case is immunoglobulin A vasculitis (IgAV), formerly named Henoch-Schönlein Purpura. With unknown etiology, this necrotizing small-vessel vasculitis is the most common form of vasculitis in children aged younger than 10 years. IgAV is seen more frequently in males, White individuals, and those of Asian descent. It is characterized by IgA-immune complex, C3, and fibrin deposition in small vessels: primarily capillaries, postcapillary venules, and occasionally arterioles in affected organs. Almost all patients develop palpable purpura. Other skin involvement may include petechiae, bullae, edema, and necrosis. Joint abnormalities are the second most common symptom and may accompany skin eruptions with severe pain and sometimes swelling, warmth, and tenderness. Ankles and knees are most often involved; symptoms are often transient and migratory.

Severe abdominal pain, vomiting, hematemesis, diarrhea, and hematochezia occur in about 50% of children, and renal involvement occurs in 20%-50% of children but is usually self-limited.

What to Look For

  • Episodes can often be preceded by an upper respiratory infection and thus are more common in the fall and winter seasons
  • The classic tetrad of symptoms are palpable purpura, abdominal pain, arthritis, and kidney disease
  • Prodromal symptoms of fever, malaise, headache, and arthralgias may be present
  • No evidence of thrombocytopenia or coagulopathy

Pearls for Urgent Care Management

  • A typical episode may persist for 3-6 weeks with spontaneous recovery, but recurrences may happen
  • Treatment includes ensuring good oral intake and pain management with nonsteroidal anti-inflammatory drugs and acetaminophen
  • Hospitalization is needed for severe disease: severe pain, decreased oral intake, kidney insufficiency, or other complications

Download the Article PDF: 15-Year-Old With Fever

15-Year-Old With Fever