Frayha R A
Clin Rheumatol. 1983 Sep;2(3):277-84. doi: 10.1007/BF02041403.
A patient with papular mucinosis (scleromyxedema) developed an erosive seronegative rheumatoid-like arthropathy, carpal tunnel syndrome, sicca complex, and marked increase in TG(OKT8) suppressor/cytotoxic circulating T-cells akin to that reported in scleroderma. Sclerodactyly, acrolysis and stiff digits were striking but other features of scleroderma, i.e., Raynaud's and esophageal hypoperistalsis, were absent. The diagnosis of papular mucinosis, a pseudoscleroderma syndrome, should be considered in a patient with atypical arthritis, median neuropathy, myopathy, and/or sclerodactyly and a papular lichenoid dermatopathy. Skin mucin stain and the demonstration of the distinct serum paraprotein (PM-spike) are confirmatory. We stress the salient diagnostic clinical features of the leonine-like facies of papular mucinosis.
一名丘疹性粘蛋白病（硬化性粘液水肿）患者出现了糜烂性血清阴性类风湿样关节炎、腕管综合征、干燥综合征，且循环中TG(OKT8)抑制/细胞毒性T细胞显著增加，类似于硬皮病中报道的情况。硬指、肢端溶解和手指僵硬很明显，但硬皮病的其他特征，即雷诺现象和食管蠕动减弱则不存在。对于患有非典型关节炎、正中神经病变、肌病和/或硬指以及丘疹性苔藓样皮肤病的患者，应考虑丘疹性粘蛋白病这一假性硬皮病综合征的诊断。皮肤粘蛋白染色和独特血清副蛋白（PM峰）的显示具有确诊意义。我们强调了丘疹性粘蛋白病狮面样面容的突出诊断临床特征。
