[Extracorporeal photopheresis--treatment option in scleromyxedema?]

Scleromyxedema is an uncommon disease of unclear etiology. Therapy is difficult. Two patients with scleromyxedema were treated with extracorporeal photopheresis (ECP). The first patient has been treated unsuccessfully for 3 months with PUVA-bath-therapy and for one year with cyclophosphamide and prednisolone. Thus supplementary treatment with ECP was initiated, as the cyclophosphamide and prednisolone were gradually reduced. After 29 cycles of ECP, the skin lesions had almost disappeared and the associated myopathy also resolved. In the second patient initial monotherapy with ECP was started after PUVA-bath-therapy for 3 months did not show any effect. After temporary improvement with ECP every four weeks, the skin lesions relapsed, so oral cyclophosphamide was added. These two cases confirm the effect of ECP in scleromyxedema, but probably combination therapy is at least initially more successful.