History: A 9 yr child with pruritic and painful rashes on the lower extremities and hands. Arthritis and abdominal pain were also noted. What’s the diagnosis?
1. IgA Vasculitis
2. Hypersensitivity vasculitis
3. Leukocytoclastic vasculitis
4. Meningococcemia
5. Rickettsial diseases
Answer: Henoch Schonlein purpura (HSP) also known as IgA vasculitis is an acute IgA mediated disorder characterized by a generalized vasculitis involving the small vessels of the skin, GI tract, kidneys, joints, and, rarely, the lungs and CNS. IgAV is the most common vasculitis in childhood. The disease is more benign in children than in adults.
Signs & Symptoms: Purpura, arthritis, and abdominal pain are known as the “classic triad” of Henoch–Schönlein purpura.
Headache, Anorexia, Fever, Rash (95-100% of cases), especially involving the legs; this is the hallmark of the disease, Abdominal pain and vomiting (35-85%), Joint pain (60-84%), especially involving the knees and ankles, Subcutaneous edema (20-50%), Scrotal edema (2-35%) or Bloody stools.
Diagnosis: Urinalysis, KFT, CBC, PT-INR, aPTT, Biopsy
DDx: IgAV may mimic an abdominal emergency, Papular urticaria, SLE, meningococcemia, dermatitis herpetiformis.
Treatment: Combination of IV methylprednisolone (steroid), cyclophosphamide and dipyridamole followed by prednisone. IVIG.