Perianal presentation of Langerhans cell histiocytosis

Abstract

Langerhans cell histiocytosis (LCH) is a rare and heterogeneous disease caused by clonal proliferation of Langerhans cells which are one of the members of the monocyte macrophage system. The incidence of LCH in adults is extremely rare: 1-2/million. Symptoms include dermatological lesions, typically seen as seborrheic-like dermatitis of the scalp, in addition to petechial lesions in intertriginous areas. Perianal ulcerative lesions are an extremely rare type of skin manifestation. Herein, we report a case of perianal presentation of LCH in an adult patient.