Tsuji T, Imajo Y, Sawabe M, Kuniyuki S, Ishii M, Hamada T, Ishimura E, Hamada N, Nishisawa Y, Morii H
Arch Dermatol. 1987 Sep;123(9):1211-6.
A 41-year-old woman developed laxity and wrinkling of the skin. This process spread slowly to involve the skin of almost the entire body, without any previous inflammatory skin disorder. The skin of her face, neck, and trunk hung in loose folds, leading to a prematurely aged appearance. One year later she noticed edema on her face and legs. Laboratory studies disclosed low C3 and CH50 serum levels and proteinuria. Skin biopsy specimens revealed extensive loss and fragmentation of dermal elastic fibers. A renal biopsy specimen showed membranoproliferative glomerulonephritis (type 2), and an immunofluorescent study disclosed C3 and IgG deposition in the mesangial matrix and along the glomerular basement membrane. We propose that, in this case, cutis laxa may be related to an abnormal immune response.
一名41岁女性出现皮肤松弛和皱纹。此过程缓慢蔓延,累及几乎全身皮肤,之前无任何炎症性皮肤病史。她面部、颈部和躯干的皮肤松弛下垂,导致过早衰老的外观。一年后,她注意到面部和腿部出现水肿。实验室检查显示血清C3和CH50水平降低以及蛋白尿。皮肤活检标本显示真皮弹性纤维广泛缺失和断裂。肾活检标本显示为膜增生性肾小球肾炎(2型),免疫荧光研究显示C3和IgG沉积于系膜基质和沿肾小球基底膜。我们认为,在该病例中,皮肤松弛可能与异常免疫反应有关。