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伪装成儿童线状IgA大疱性皮肤病的弥漫性皮肤肥大细胞增多症。

Diffuse cutaneous mastocytosis masquerading as linear IgA bullous dermatosis of childhood.

作者信息

Rayinda Tuntas, Oktarina Dyah Ayu Mira, Danarti Retno

机构信息

Department of Dermatology and Venereology, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Yogyakarta, Indonesia.

出版信息

Dermatol Reports. 2021 Mar 17;13(1):9021. doi: 10.4081/dr.2021.9021. eCollection 2021 Mar 18.

Abstract

Diffuse cutaneous mastocytosis is a rare form of cutaneous mastocytosis that can appear in heterogeneous clinical presentations, including eruption of papules, erythematous plaques, blisters, and erythroderma. We report a 1.5- year-old boy who presented with itchy wheals and blisters spreading on his body. The patient was initially managed as a linear IgA bullous dermatosis of childhood (LABD) because of the similarity of clinical symptoms and the presenting of linear IgA deposits at the basement membrane. Due to the development of urticarial plaque after the resolution of the blisters, the diagnosis of diffuse cutaneous mastocytosis was made based on clinical, histopathological (hematoxylin-eosin, Giemsa, and toluidine blue staining), and direct immunofluorescent examinations (IgA, IgG, IgM, C3). The symptoms were improved following antihistamines and oral corticosteroid treatment.

摘要

弥漫性皮肤肥大细胞增多症是皮肤肥大细胞增多症的一种罕见形式,可表现为多种不同的临床表现,包括丘疹、红斑性斑块、水疱和红皮病。我们报告一名1.5岁男孩,其身上出现瘙痒性风团和水疱并蔓延。由于临床症状相似且在基底膜出现线性IgA沉积,该患者最初被诊断为儿童线状IgA大疱性皮肤病(LABD)。水疱消退后出现荨麻疹性斑块,基于临床、组织病理学(苏木精-伊红、吉姆萨和甲苯胺蓝染色)及直接免疫荧光检查(IgA、IgG、IgM、C3)确诊为弥漫性皮肤肥大细胞增多症。使用抗组胺药和口服糖皮质激素治疗后症状改善。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c297/8018259/f62574cf45d3/dr-13-1-9021-g001.jpg

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