Peter L M, Ammoury A, Chiavassa-Gandois H, Lamant L, Paul C F
Department of Dermatology, Purpan Hospital and Paul Sabatier University, Toulouse, France.
Clin Exp Dermatol. 2008 Aug;33(5):606-10. doi: 10.1111/j.1365-2230.2008.02744.x. Epub 2008 May 12.
We report a patient with scleromyxoedema and peripheral neuropathy treated successfully with thalidomide. An objective evaluation was carried out using histopathology, cutaneous ultrasonography and magnetic resonance imaging (MRI). A 67-year-old woman presented with a leonine face, generalized thickened skin, an underlying peripheral neuropathy and a monoclonal gammopathy. She was treated with thalidomide 100 mg/day. After 20 months of therapy, there was a dramatic clinical improvement in the skin lesions, and the neuropathy also improved. Cutaneous ultrasonography showed a reduction in dermal thickness, whereas the results of the cutaneous MRI were inconclusive. Thalidomide appears to be effective in scleromyxoedema. Its specific effect on the underlying monoclonal gammopathy might have contributed to the improvement in the skin and neurological symptoms. In this case, assessment of cutaneous improvement with cutaneous ultrasonography was superior to that of cutaneous MRI. Thalidomide should be considered for the treatment of scleromyxoedema despite the presence of an underlying peripheral neuropathy.
我们报告了一例使用沙利度胺成功治疗的硬化性黏液水肿合并周围神经病变患者。采用组织病理学、皮肤超声和磁共振成像（MRI）进行了客观评估。一名67岁女性，表现为狮面、全身皮肤增厚、潜在的周围神经病变和单克隆丙种球蛋白病。她接受了每日100mg沙利度胺的治疗。治疗20个月后，皮肤病变有显著临床改善，神经病变也有所改善。皮肤超声显示真皮厚度减小，而皮肤MRI结果尚无定论。沙利度胺似乎对硬化性黏液水肿有效。其对潜在单克隆丙种球蛋白病的特定作用可能有助于皮肤和神经症状的改善。在本病例中，用皮肤超声评估皮肤改善情况优于皮肤MRI。尽管存在潜在的周围神经病变，沙利度胺仍应被考虑用于治疗硬化性黏液水肿。
