Martin Laimer, Konrad Namberger, Cesare Massone, Josef Koller, Michael Emberger, Lisa Pleyer, Helmut Hintner, Richard Greil
DOI: 10.2340/00015555-0731

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 thera peutic trials. This paper discusses current proposed therapeutic strategies and reports the case of a 64-year- ld male patient with progressive scleromyxoedema associated with IgG- 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.