Multisystem Langerhans cell histiocytosis: successful treatment with thalidomide

A 57-year-old woman presented with a 1-year history of non-healing inguinal and perianal ulcers as well as a 2-year history of polydipsia. The clinical picture and histology suggested Langerhans cell histiocytosis, which was confirmed by immunohistochemistry and electron microscopy. Magnetic resonance imaging of the brain revealed hypothalamic-pituitary axis involvement. Treatment was started with thalidomide and desmopressin. The skin lesions resolved over 2 months. Over the next 24 months of continued therapy, the cerebral involvement remained stable and the skin lesions did not recur.