Sillard L, Passeron T, Cardot-Leccia N, Perrin C, Lacour J-P, Ortonne J-P
Service de dermatologie, hôpital Archet 2, CHU de Nice, route de St-Antoine-de-Ginestière, 06200 Nice, France.
Ann Dermatol Venereol. 2010 Jan;137(1):48-52. doi: 10.1016/j.annder.2009.11.010. Epub 2009 Dec 29.
Scleromyxoedema is a rare disorder of unknown pathogenesis that is very difficult to treat. We report a case resistant to corticosteroid treatment but controlled by intravenous gammaglobulin (IVIG).
A 60-year-old woman presented progressive generalized papular eruption with infiltrated and itchy lesions of between 2 and 5mm in diameter. Otherwise, the clinical examination was normal. Monoclonal gammopathy of the IgG lambda type was found. Histology confirmed the diagnosis of scleromyxoedema. The disease continued to progress despite oral corticosteroids (0.5mg/kg per day). Thalidomide was introduced but was discontinued after 2 months due to side effects. Treatment comprising six monthly infusions of IVIG (2g/kg on 5 days) resulted in a marked reduction (>50%) in lesions. Two months after discontinuation of IVIG, recurrence was observed and maintenance infusions of IVIG every 6 weeks were needed to control the disease.
The course of scleromyxoedema is unpredictable and treatment is extremely difficult. Successful therapy with IGIV has been reported but this approach seems to afford only temporary relief and maintenance infusions are required, as confirmed by the initial efficacy of treatment in our patient and the rapid recurrence of lesions following withdrawal.
硬化性黏液水肿是一种发病机制不明的罕见疾病，极难治疗。我们报告一例对皮质类固醇治疗耐药但通过静脉注射丙种球蛋白（IVIG）得到控制的病例。
一名60岁女性出现进行性全身性丘疹性皮疹，有直径2至5毫米的浸润性且瘙痒性皮损。除此之外，临床检查正常。发现有IgGλ型单克隆丙种球蛋白病。组织学检查确诊为硬化性黏液水肿。尽管口服皮质类固醇（每天0.5mg/kg），病情仍继续进展。使用了沙利度胺，但因副作用在2个月后停药。包括每6个月静脉注射一次IVIG（5天内2g/kg）的治疗使皮损明显减少（>50%）。停用IVIG两个月后，观察到复发，需要每6周进行一次IVIG维持输注以控制病情。
硬化性黏液水肿的病程不可预测，治疗极其困难。已有使用静脉注射免疫球蛋白（IGIV）成功治疗的报道，但正如我们患者治疗的初始疗效以及停药后皮损迅速复发所证实的那样，这种方法似乎只能提供暂时缓解，需要维持输注。
