Langerhans cell histiocytosis in adult patients: cutaneous and mucous localizations improving with thalidomide

Background. - Langerhans cell histiocytosis is a rare disorder with a wide spectrum of diseases and various clinical behaviours. The prognosis depends on the patient's age at onset and the extension of the disease. Treatment is function of Evolution and extension of the disease. Case report. - We report one case of Langerhans cell histiocytosis in a 33-year-old woman with resistant vulvar involvement whose cutaneous lesions improved with thalidomide. Complete healing of the genital lesions occurred within 1 month, but the patient discontinued her treatment after 3 months because of somnolence. Recurrence of the symptoms was observed within 2 weeks without treatment, but complete control was obtained with reintroduction of thalidomide. No severe adverse effect was detected at electrophysiological and clinical examinations. Conclusion. - The simplicity and the rapid effect thalidomide on cutaneous lesions suggest that new clinical evaluation of this old drug that can also be used in some systemic disease might be of the value. (C) 1998, Elsevier. Paris.