Department of Dermatology, Paracelsus Medical University Salzburg, Salzburg, Austria.
Acta Derm Venereol. 2009 Nov;89(6):631-5. 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 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.
硬肿性黏液水肿是一种病因不明的罕见疾病，其特征为进行性皮肤黏液水肿和副蛋白血症。多种全身性（例如胃肠道、神经、肺、心脏和肾脏）并发症可能导致严重的发病率和死亡率，需要进行治疗干预。后者仍然具有挑战性。文献中报道了多种治疗方法，但往往反应不一致、频繁复发且可能有严重的副作用。此外，硬肿性黏液水肿的罕见性使得无法进行对照治疗试验。本文讨论了当前提出的治疗策略，并报告了一例 64 岁男性患者的病例，该患者患有进行性硬肿性黏液水肿，伴有 IgG-λ 副蛋白血症，在 12 周内，每月给予长春新碱、柔红霉素和地塞米松联合每日口服沙利度胺治疗，实现了临床和实验室缓解。
