Remission of scleromyxoedema following treatment with extracorporeal photopheresis.
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.
Krasagakis
K
K
Department of Dermatology, Free University of Berlin, Germany.
Zouboulis
C C
CC
Owsianowski
M
M
Ramaker
J
J
Trautmann
C
C
Tebbe
B
B
Orfanos
C E
CE
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
D007074:Immunoglobulin G