Scleromyxedema: complete response to extracorporeal photopheresis and steroids.

Introduction, Scleromyxedema is the consequence of a dermal infiltration by mucine, commonly associated with a monoclonal gammapathy of unknown signif icance. Case report. A 46 year-old woman was hospitalized for a scleromyxedema with a bilateral macular edema and a restricted pulmonary syndrome. A quite com plete cutaneous response and a complete ocular and pulmonary response were obtained after 12 extracorporeal photopheresis courses and 4 flashes of pre dnisolone (17 months follow-up). Comments, There is no consensus on guidelines for the treatment of scleromy xedema. Steroids and melphalan are usually indicated. However these drugs i nduce severe side-effects. In the absence of controlled studies concerning the efficiency of the different drugs used in scleromyxedema, the main adva ntage of photopheresis lies in the safety of this procedure.