Vincristine, Idarubicin, Dexamethasone and Thalidomide in Scleromyxoedema

Authors

  • Martin Laimer
  • Konrad Namberger
  • Cesare Massone
  • Josef Koller
  • Michael Emberger
  • Lisa Pleyer
  • Helmut Hintner
  • Richard Greil

DOI:

https://doi.org/10.2340/00015555-0731

Keywords:

chemotherapy, paraproteinaemia, mucinosis.

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 thera peutic 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.

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Published

2009-09-21

How to Cite

Laimer, M., Namberger, K., Massone, C., Koller, J., Emberger, M., Pleyer, L., Hintner, H., & Greil, R. (2009). Vincristine, Idarubicin, Dexamethasone and Thalidomide in Scleromyxoedema. Acta Dermato-Venereologica, 89(6), 631–635. https://doi.org/10.2340/00015555-0731

Issue

Section

Articles