Widmer M, Reverdin E, Küng E, Vesti B, Bollinger A, Linke R P, Burg G
Dermatologische Klinik, Universitätsspital Zürich.
Hautarzt. 1993 May;44(5):319-23.
We report an exceptionally severe case of amyloidosis cutis nodularis atrophicans (Gottron) of the lower limbs in a 49-year-old female patient. The diagnosis was made on clinical and histological grounds and was confirmed by immunohistochemical evidence of amyloid of the A-lambda type. For the first time, cutaneous microcirculation in the region of the nodules was investigated by means of fluorescence video microscopy. Pronounced microangiopathy characterized by avascular fields, enlarged and tortuous capillaries and increased transcapillary diffusion of sodium fluorescein, was clearly demonstrable in the area of the nodules. The pathogenetic role of these vascular changes is discussed.
我们报告了一例49岁女性患者下肢的萎缩性结节性皮肤淀粉样变(Gottron型)的极其严重的病例。诊断基于临床和组织学依据,并通过A-λ型淀粉样蛋白的免疫组化证据得以证实。首次通过荧光视频显微镜对结节区域的皮肤微循环进行了研究。在结节区域可清楚地显示出以无血管区、毛细血管扩张迂曲以及荧光素钠经毛细血管扩散增加为特征的明显微血管病变。文中讨论了这些血管变化的致病作用。