Adult cutaneous Langerhans cell histiocytosis

A 24-year-old male presented with a 4 year history of a crusted erythematous papular eruption of the scalp and external auditory meati and a 12 month history of painful perianal ulceration. A diagnosis of Langerhans cell histiocytosis was made and confirmed by skin histology. Extensive investigation revealed no systeme involvement. rapid improvement occured after intravenous 2--chlorodeoxyadenosine but relapse of perianal lesions occured within 3 months. local radiotherapy to the perianal region resulted in a complete remission sustained over 12 months.