WYK Chan, RYY Leung, AKS Chiang, DKL Cheuk

Abstract

Blastic plasmacytoid dendritic cell neoplasm is an uncommon aggressive haemato-lymphoid neoplasm derived from type 2 dendritic cells which occur mainly in adults and elderly. It usually manifests as cutaneous lesions on face, scalp, trunk or limbs. In this report, we describe a 6-year old girl with unusual presentation of septic shock and perianal necrotising fasciitis, warranting urgent laparotomy, colectomy, perineal debridement and long term colostomy. She achieved complete remission with acute lymphoblastic leukaemia-based chemotherapy protocol.