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[伴有肢端角化病的弥漫性皮肤异色症,先有大疱性损害:韦里-金德勒病。分类学和组织病理学讨论]

[Diffuse poikiloderma with acrokeratosis, preceded by bullous lesions: Weary-Kindler's disease. Nosologic and histopathologic discussion].

作者信息

Maleville J, Cavaroc Y, Boiron G, Tamisier J M, Surlève-Bazeille J E, Larrègue M, Sarrat P, Guillet G

出版信息

Ann Dermatol Venereol. 1982;109(11):949-56.

PMID:6762840
Abstract

The authors report the case of a six and a half year old girl, with extensive poikilodermatous condition and acrokeratosis, preceded by early blistering and light sensitivity. The sequence of cutaneous symptoms suggests the diagnosis of hereditary and bullous acrokeratotic poikiloderma of Weary and Kindler; forty-two cases have already been reported in the literature and our patient would better fit into Kindler's type. The original findings under electron microscopy and direct immunofluorescence (colloid bodies, IgM deposition) that are reported herein are discussed from a pathogenic point of view.

摘要

作者报告了一例6岁半女童的病例,该女童患有广泛的皮肤异色症和肢端角化病,之前有早期水疱形成和光敏感症状。皮肤症状的先后顺序提示诊断为Weary和Kindler遗传性大疱性肢端角化性皮肤异色症;文献中已报道42例,我们的患者更符合Kindler型。本文从发病机制的角度讨论了在此报告的电子显微镜和直接免疫荧光检查的原始发现(胶样小体、IgM沉积)。

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