Childhood polyarteritis nodosa: A rare presentation

Abstract

Polyarteritis nodosa (PAN) is a rare systemic vasculitis characterized by necrotizing arteritis of small to medium-sized arteries. It manifests as skin ulceration, hypertension, abdominal pain, digital gangrene, subcutaneous nodules etc. Childhood polyarteritis nodosa (CPAN) is a rare and often fatal disease. Clinically, CPAN often is part of the spectrum of Kawasaki disease (KD). There is no specific serological marker for diagnosis and confirmation is based on histopathology. Most common mode of pharmacological therapy includes corticosteroid or cyclophosphamide, along with antimicrobials for secondary infection prevention and plasmapheresis. Here, we present a case of a 16year old boy with multiple skin ulceration, digital gangrene with few non-specific signs and symptoms, diagnosed as a case of PAN, underwent regular therapy and follow-up.

Keywords

• childhood polyarteritis nodosa
• corticosteroids
• mycophenolate mofetil