Adult Langerhans Cell Histiocytosis Limited to the Skin

Abstract
Background: The incidence of Langerhans cell histiocytosis (LCH) is 4–5 per million in children with only 30% of this number having an adult onset. While dermatological manifestations occur in as many as 50% of cases, disease limited to the skin is uncommon among reported cases of adult LCH. Objectives and Methods: To present 3 new cases of adult LCH and a review of the literature of isolated cutaneous LCH in adults. Results: Three adults with scalp, vulvar and generalized LCH lesions had refractory responses to treatment. Conclusions: LCH may present with unusual cutaneous manifestations limited to the skin in adults. Optimal treatment has not yet been determined.