title: Remission of scleromyxoedema following treatment with extracorporeal photopheresis.
abstract: Scleromyxoedema, a disseminated papular and sclerotic variant of lichen myxoedematosus, is a rare disease with a chronic progressive course, and little tendency towards spontaneous remission. The treatment of scleromyxoedema has been largely ineffective. Aggressive chemotherapeutic agents have been used, often leading to therapy-related morbidity and mortality. We report a 41-year-old woman with scleromyxoedema, associated with a monoclonal gammopathy of IgG-kappa type, whose condition almost completely cleared with 12 monthly sessions of extracorporeal photopheresis. The patient had previously not responded to isotretinoin, and chlorambucil with prednisolone.
authors.affiliation: Department of Dermatology, Free University of Berlin, Germany.
authors.forename: K
authors.initials: K
authors.lastname: Krasagakis
authors.forename: C C
authors.initials: CC
authors.lastname: Zouboulis
authors.forename: M
authors.initials: M
authors.lastname: Owsianowski
authors.forename: J
authors.initials: J
authors.lastname: Ramaker
authors.forename: C
authors.initials: C
authors.lastname: Trautmann
authors.forename: B
authors.initials: B
authors.lastname: Tebbe
authors.forename: C E
authors.initials: CE
authors.lastname: Orfanos
mesh_terms: D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D017512:Lichenoid Eruptions / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology; D017520:Mucinoses / Q000188:drug therapy* / Q000209:etiology / Q000473:pathology; D010265:Paraproteinemias / Q000150:complications; D017893:Photopheresis
chemical_list: D007074:Immunoglobulin G