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Esther von Stebut, MD; Simin Schadmand-Fischer, MD; Wolfgang Bräuninger, MD; Andreas Kreft, MD; Claus Doberauer, MD; Kerstin Steinbrink, MD

Arch Dermatol. 2008;144(5):649-653.

Background  Langerhans cell histiocytosis (LCH) is a rare disease with a peak incidence in childhood. There is limited experience with treatment options for adult patients having multisystemic LCH involvement. We report successful treatment of a 70-year-old woman with adult onset of LCH and multisystem disease (diabetes insipidus centralis, bone marrow infiltration, and lung and skin involvement).

Observations  A 70-year-old woman with erythematous plaques and papules of the submammary and inguinal skin attended our outpatient clinic and was diagnosed as having LCH. Organ involvement was found in the infundibulum of the pituitary gland, associated with diabetes insipidus centralis, bone marrow infiltration, and several micronodules of the thoracic and lumbar spine and lungs. Based on the Histiocyte Society's LCH-A1 study in adults, the patient was treated for 12 months with a combination of corticosteroids, vinblastine, and mercaptopurine. No major adverse effects were observed. The skin was also treated with a combination of psoralen–UV-A and local corticosteroids. Restaging revealed regression of all clinical symptoms (skin involvement and diabetes insipidus centralis) and regression of organ infiltration (pituitary gland, bone marrow, and lungs).

Conclusion  Effective treatment of adult multisystemic LCH disease was achieved using prednisolone, vinblastine, and mercaptopurine, which was well tolerated.

Author Affiliations: Departments of Dermatology (Drs von Stebut, Bräuninger, and Steinbrink) and Radiology (Dr Schadmand-Fischer) and Institute of Pathology (Dr Kreft), University of Mainz, Mainz, and Department of Internal Medicine, Evangelische Kliniken Gelsenkirchen, Gelsenkirchen (Dr Doberauer), Germany.

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