Adult cutaneous Langerhans’ cell histiocytosis: a rare presentation, successful treatment with thalidomide

Abstract

Langerhans’ cell histiocytosis (LCH) is a rare clonal disorder of proliferating histiocytic cells  expressing phenotypic markers of the epidermal Langerhans’ cells. LCH generally affects  children. Adult LCH with single system disease limited to skin is uncommon and difficult to  diagnose. The management of LCH is difficult as these disorders respond inconsistently to  immunosuppressive and chemotherapeutic strategies. We report a case of 48 year female,  diabetic and hypertensive presented as single system LCH limited to skin with history of  recurrent painful erythematous ulcerated lesions in right axilla and both groins since 4 years  with and limiting mobility of limbs. She was successfully treated with thalidomide.  Thalidomide monotherapy represents an effective, safe and well-tolerated treatment option  that should be considered as first-line therapy for single system LCH limited to skin which is  rare and difficult to treat.