Churg-Strauss증후군 치험 1예

Abstract

Churg-Strauss syndrome is one of idiopathic, systemic necrotizing vasulitides and characterized by a history of asthma or allergic rhinitis, eosinophilia of peripheral blood and variable symptoms of the systemic vasculitis. This is a case report of classical Churg-Strauss syndrome in a 6- year-old male who has the multiple mononeuropathy as a symptom of the systemic vasculitis. The sural nerve biopsy showed a few myelin digestion chambers suggesting axonal degeneration which was not suspected on the electrophysiological study, and the specimen of right gastrocnemius muscle revealed the typical pathological findings of eosinophilic infiltration, extravascular granuloma and necrotizing small vessel vasculitis. The therapeutic response of cyclophosphamide looked better than the oral prednisolone treatment only.