title: Vincristine, idarubicin, dexamethasone and thalidomide in scleromyxoedema.
abstract: Scleromyxoedema is a rare disease of unknown aetiology that is characterized by progressive cutaneous mucinosis and paraproteinaemia. A variety of systemic (e.g. gastro intestinal, neurological, pulmonary, cardiac and renal) complications may lead to significant morbidity and mortality necessitating therapeutic intervention. The latter remains challenging. Numerous treatment modalities have been reported in the literature, often, however, with inconsistent responses, frequent relapses and potentially serious side-effects. Moreover, the rarity of scleromyxoedema has prevented the execution of controlled therapeutic trials. This paper discusses current proposed therapeutic strategies and reports the case of a 64-year-old male patient with progressive scleromyxoedema associated with IgG-lambda paraproteinaemia in whom monthly administrations of vincristine, idarubicin and dexamethasone in addition to daily oral thalidomide led to clinical and laboratory remission within 12 weeks.
authors.affiliation: Department of Dermatology, Paracelsus Medical University Salzburg, Salzburg, Austria. m.laimer@salk.at
authors.forename: Martin
authors.initials: M
authors.lastname: Laimer
authors.forename: Konrad
authors.initials: K
authors.lastname: Namberger
authors.forename: Cesare
authors.initials: C
authors.lastname: Massone
authors.forename: Josef
authors.initials: J
authors.lastname: Koller
authors.forename: Michael
authors.initials: M
authors.lastname: Emberger
authors.forename: Lisa
authors.initials: L
authors.lastname: Pleyer
authors.forename: Helmut
authors.initials: H
authors.lastname: Hintner
authors.forename: Richard
authors.initials: R
authors.lastname: Greil
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D003907:Dexamethasone / Q000627:therapeutic use; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D006801:Humans; D015255:Idarubicin / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000150:complications; D012074:Remission Induction; D053718:Scleromyxedema / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D013792:Thalidomide / Q000627:therapeutic use; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D000970:Antineoplastic Agents; D007166:Immunosuppressive Agents; D013792:Thalidomide; D014750:Vincristine; D003907:Dexamethasone; D015255:Idarubicin