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Vincristine, Idarubicin, Dexamethasone and Thalidomide in Scleromyxoedema
- Source :
- Acta Dermato Venereologica. 89:631-635
- Publication Year :
- 2009
- Publisher :
- Medical Journals Sweden AB, 2009.
-
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.
- Subjects :
- Male
Vincristine
medicine.medical_specialty
medicine.medical_treatment
Paraproteinemias
Antineoplastic Agents
Dermatology
Dexamethasone
Drug Administration Schedule
Pharmacotherapy
medicine
Humans
Idarubicin
Antibacterial agent
Chemotherapy
business.industry
Remission Induction
General Medicine
Middle Aged
Thalidomide
Surgery
Scleromyxedema
Drug Therapy, Combination
business
Immunosuppressive Agents
medicine.drug
Rare disease
Subjects
Details
- ISSN :
- 00015555
- Volume :
- 89
- Database :
- OpenAIRE
- Journal :
- Acta Dermato Venereologica
- Accession number :
- edsair.doi.dedup.....9c078894c4924318c4a4bceb17f94cb8