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迟发性皮肤卟啉症(PCT)的皮肤损伤形态学

[The morphology of skin damage in porphyria cutanea tarda (PCT)].

作者信息

Kemmer C, Reitmann I, Köstler E, Riedel H

机构信息

Institut für Pathologische Anatomie, Medizinischen Akademie Carl Gustav Carus Dresden, DDR.

出版信息

Zentralbl Allg Pathol. 1988;134(3):227-34.

PMID:3055747
Abstract

Skin biopsies (fresh and old blisters) of the back of the hand and lower arm, respectively, obtained from 24 patients with clinically and biochemically established porphyria cutanea tarda have been examined light and electron microscopically, 15 out of them also by immunofluorescence microscopy (occurrence of IgG, IgM and C3). Light microscopically there were subepidermal blisterings and typical corial broadenings of the capillary wall with deposits of diastase-resistant PAS-positive material in and around the walls of the vessels. Electron microscopically, replications of the capillary basement membrane with deposits of amorphous material were observed. IgG and C 3 on the dermoepidermal junction and in the capillaries could be identified by means of the direct immunofluorescence method in a number of cases. The pathogenesis of the lesions of the vessel walls and that of the subepidermal blistering, hitherto interpreted as their consequence, is not yet clarified sufficiently.

摘要

分别从24例临床和生化确诊的迟发性皮肤卟啉病患者的手背和下臂获取皮肤活检样本(新鲜水疱和陈旧水疱),进行光镜和电镜检查,其中15例还进行了免疫荧光显微镜检查(检测IgG、IgM和C3的存在情况)。光镜下可见表皮下水疱形成,以及典型的真皮层毛细血管壁增宽,血管壁内及周围有抗淀粉酶PAS阳性物质沉积。电镜下观察到毛细血管基底膜重复出现并有无定形物质沉积。在一些病例中,通过直接免疫荧光法可在真皮表皮交界处和毛细血管中鉴定出IgG和C3。血管壁病变的发病机制以及迄今为止被认为是其后果的表皮下水疱形成的发病机制,尚未得到充分阐明。

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