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Case Reports

Abstract

Acquired factor V inhibitor is a rare hematological condition characterized by the development of autoantibodies targeting coagulation factor V, which typically presents with increased bleeding. Herein, we describe a perplexing case of acquired factor five inhibitor with lupus-like features which clinically presented as Fournier gangrene/necrotizing fasciitis. Following a hemorrhoidectomy procedure, a 69-year-old male presented with priapism. Physical exam revealed dusky discoloration of the perineum, and a biopsy was performed due to clinical concern for necrotizing fasciitis. The biopsy unveiled unexpected leukocytoclastic vasculitis and thrombotic vasculopathy and a coagulopathy work-up was suggested to the clinical team. Notably, cultures obtained from the necrotic tissue failed to yield any microbial growth. Subsequent coagulation studies demonstrate elevated APTT, PT, INR, decreased factor V, and the areas of cutaneous necrosis continued to progress. This case highlights the importance of maintaining a comprehensive range of differential diagnoses and the importance of clinicopathologic correlation and communication.