儿童IgA天疱疮1例报告

Serbian Journal of Dermatology and Venereology Pub Date : 2017-03-01 DOI:10.1515/sjdv-2017-0003

B. Lekić, M. Gajić‐Veljic, S. Popadic, M. Nikolic

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引用次数: 0

摘要

摘要IgA天疱疮（IGAP）是一种罕见的自身免疫性大疱性疾病，其特征是IgA沉积在角质形成细胞表面。IGAP分为：1）角膜下脓疱性皮肤病（SPD）型和2）表皮内嗜中性粒细胞（IEN）IgA皮肤病型。到目前为止，文献中只描述了9例IGAP儿童，其中只有3例为SPD型。我们报告一名患有SPD型IGAP的3岁男孩。临床上，他表现为面部、躯干、腹股沟和四肢的瘙痒性囊泡、脓疱和糜烂。组织病理学显示角膜下脓疱含有少量棘皮细胞。Tzanck涂片的直接免疫荧光（DIF）试验显示，细胞间IgA沉积在表皮细胞组的表面。口服氨苯砜和泼尼松两周后病情缓解；11个月后停止治疗，19个月内完全缓解，无需任何治疗。Tzanck涂片直接免疫荧光法是一种简单、灵敏、快速、无攻击性的检测方法，非常适合于儿童IGAP的诊断。

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IgA Pemphigus in a Child – a Case Report

Abstract IgA pemphigus (IGAP) is a rare autoimmune bullous disease characterized by IgA deposits on keratinocyte cell surfaces. The IGAP is classified into: 1) subcorneal pustular dermatosis (SPD) type, and 2) intraepidermal neutrophilic (IEN) IgA dermatosis type. So far, only 9 children with IGAP have been described in the literature, of whom only 3 with SPD type. We report a 3-year-old boy with SPD type of IGAP. Clinically, he presented with pruritic vesicles, pustules and erosions on the face, trunk, groin area, and extremities. Histopathology showed subcorneal pustules containing a few acantholytic cells. Direct immunofluorescence (DIF) test of Tzanck smear showed intercellular IgA deposits on the surface of the groups of epidermal cells. Oral dapsone and prednisone induced remission after two weeks; the treatment was discontinued 11 months later, and complete remission was achieved during 19 months without any treatment. Direct immunofluorescence of Tzanck smear is a simple, sensitive, rapid and non-aggressive test, very suitable for the diagnosis of IGAP in children.

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来源期刊

Serbian Journal of Dermatology and Venereology

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期刊介绍： Serbian Journal of Dermatology and Venereology is a journal of the Serbian Association of Dermatologists and Venereologists. The journal is published in English, quarterly and intended to provide rapid publication of papers in the field of dermatology and venereology. Manuscripts are welcome from all countries in the following categories: editorials, original studies, review articles, professional articles, case reports, and history of medicine.