title: Successful treatment of cutaneous langerhans cell histiocytosis with thalidomide.
abstract: Langerhans cell histiocytosis (LCH) represents a group of rare histiocytic syndromes characterized by tissue infiltration with dendritic cells. The management of LCH is difficult as these disorders respond inconsistently to immunosuppressive and chemotherapeutic strategies. Thalidomide (N-phtalimidoglutarimide), initially used as a tranquilizer, has recently been used in the management of several inflammatory skin diseases. We describe the case of a 38-year-old male with mucocutaneous LCH. A treatment course with 6 cycles of 2-chlorodeoxyadenosin (cladribine) was initiated. This was well tolerated but withdrawn after 6 months to prevent secondary malignancy. A partial remission was seen. Subsequently, a treatment course with thalidomide 200 mg daily was started. This therapy resulted in a significant improvement of the mucocutaneous lesions within 4 weeks and complete healing was achieved after 3 months. Treatment was then successfully continued with daily doses of 100 mg to prevent relapse. In conclusion, thalidomide monotherapy represents an effective, safe and well-tolerated treatment option that should be considered as first-line therapy for mucocutaneous LCH.
authors.affiliation: Department of Dermatology and Allergology, Friedrich Schiller University Jena, Jena, Germany.
authors.forename: C S
authors.initials: CS
authors.lastname: Sander
authors.forename: M
authors.initials: M
authors.lastname: Kaatz
authors.forename: P
authors.initials: P
authors.lastname: Elsner
mesh_terms: D000284:Administration, Oral; D000328:Adult; D001707:Biopsy, Needle; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005500:Follow-Up Studies; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D008297:Male; D018570:Risk Assessment; D012720:Severity of Illness Index; D012871:Skin Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology*; D013792:Thalidomide / Q000008:administration & dosage*; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents; D013792:Thalidomide