Le Moigne M, Mazereeuw-Hautier J, Bonnetblanc J-M, Astudillo L, D'Incan M, Bessis D, Thomas L, Debarbieux S, Ammoury A, Lamant L, Paul C
Dermatologie, CHU de Toulouse, Université Paul-Sabatier, Toulouse, France.
Ann Dermatol Venereol. 2010 Dec;137(12):782-8. doi: 10.1016/j.annder.2010.08.011. Epub 2010 Sep 28.
scleromyxoedema is characterized by dermal mucin deposition associated with a monoclonal gammopathy. This is a rare disease, mostly reported as isolated cases. There is limited data regarding the course and prognosis of the disease. The aim of this study was to determine the clinical characteristics and course of scleromyxoedema.
this was a retrospective study in patients from five French university hospitals between 1987 and 2007. Data were collected using a standardized questionnaire. The inclusion criteria were based on the disease diagnosis criteria proposed by Rongioletti and Rebora: (1) generalized, papular and sclerodermiform skin eruption, (2) mucin deposition in the dermis, fibroblastic proliferation and skin fibrosis, (3) presence of a monoclonal gammopathy, (4) absence of thyroid disease.
eight patients were included. The mean age at disease onset was 51.5 years (range: 35-67). The mean time from primary symptoms and diagnosis was 41.6 months (range: 4-120). Seven patients had extra-cutaneous involvement: four with peripheral neuropathy and three with interstitial pneumonia. The mean follow-up time was 9 years. Four patients improved: two experienced partial remission and two complete remission. Complete remission was obtained under treatment with dexamethasone (one patient) and thalidomide (one patient). One patient presented a myeloma and one patient presented encephalopathy leading to death.
our study shows the frequency of extra-cutaneous involvement and shows that complete remission occurs in some patients.
硬化性黏液水肿的特征是皮肤黏蛋白沉积伴单克隆丙种球蛋白病。这是一种罕见疾病，大多作为散发病例报道。关于该疾病的病程和预后的数据有限。本研究的目的是确定硬化性黏液水肿的临床特征和病程。
这是一项对1987年至2007年间来自法国五所大学医院患者的回顾性研究。使用标准化问卷收集数据。纳入标准基于Rongioletti和Rebora提出的疾病诊断标准：（1）全身性、丘疹性和硬皮病样皮疹；（2）真皮内黏蛋白沉积、成纤维细胞增殖和皮肤纤维化；（3）存在单克隆丙种球蛋白病；（4）无甲状腺疾病。
纳入8例患者。疾病发病的平均年龄为51.5岁（范围：35 - 67岁）。从主要症状出现到诊断的平均时间为41.6个月（范围：4 - 120个月）。7例患者有皮肤外受累：4例有周围神经病变，3例有间质性肺炎。平均随访时间为9年。4例患者病情改善：2例部分缓解，2例完全缓解。地塞米松（1例患者）和沙利度胺（1例患者）治疗后获得完全缓解。1例患者出现骨髓瘤，1例患者出现脑病并导致死亡。
我们的研究显示了皮肤外受累的频率，并表明一些患者出现了完全缓解。
