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新生儿线状IgA大疱性皮肤病

Linear IgA bullous dermatosis in a neonate.

作者信息

Hruza L L, Mallory S B, Fitzgibbons J, Mallory G B

机构信息

Department of Pathology, Washington University School of Medicine, St. Louis, Missouri 63110.

出版信息

Pediatr Dermatol. 1993 Jun;10(2):171-6. doi: 10.1111/j.1525-1470.1993.tb00049.x.

Abstract

A newborn black boy had two facial blisters at birth that progressed to bullous lesions over the trunk, genitals, extremities, and oral and tracheal mucosa. A biopsy specimen demonstrated a subepidermal bulla with mixed eosinophilic and neutrophilic, inflammatory infiltrate. Direct immunofluorescence showed linear IgA, IgG, and C3 depositions along the basement membrane zone, consistent with a diagnosis of childhood linear IgA bullous dermatosis (chronic bullous dermatosis of childhood). The skin disease was controlled with combined prednisone and dapsone. This is the youngest reported patient with the disease. Linear IgA bullous dermatosis should be considered in the differential diagnosis of blistering diseases of the newborn, and immunofluorescence should be performed on a skin biopsy specimen.

摘要

一名新生黑人男婴出生时面部有两个水疱,随后发展为累及躯干、生殖器、四肢以及口腔和气管黏膜的大疱性损害。活检标本显示表皮下水疱,伴有嗜酸性粒细胞和中性粒细胞混合性炎性浸润。直接免疫荧光检查显示沿基底膜带呈线性IgA、IgG和C3沉积,符合儿童线状IgA大疱性皮病(儿童慢性大疱性皮病)的诊断。该皮肤病通过泼尼松和氨苯砜联合治疗得到控制。这是报道的患该疾病最年幼的患者。在新生儿水疱性疾病的鉴别诊断中应考虑线状IgA大疱性皮病,并且应对皮肤活检标本进行免疫荧光检查。

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