儿童线状IgA大疱性皮病1例

患儿男,2岁9个月。面颊、背部、双下肢泛发红斑、水疱伴疼痛15天。皮肤科情况:双侧面颊、耳廓可见红斑、糜烂,部分结痂。双下肢遍布黄豆大水疱,疱壁紧张,疱液清亮,尼氏征(-)。皮损组织病理示表皮下水疱形成,疱内可见浆液、中性粒细胞、淋巴细胞及嗜酸性粒细胞。免疫荧光IgA(+),基底膜可见线状沉积。诊断:儿童线状IgA大疱性皮病。

A Case of Linear IgA Bullous Dermatosis of Childhood

A 2-year and nine month-old boy with a sudden eruption of blisters and erythema on his face, trunk,and lower limbs with pain over 15 days was referred to our department. Medical examination:there was an eruption of erythema and anabrosis on his face and auricle. Big blisters with tense blister wall and clear blisterfluid,which Nikolsky,s sign were negative,wildly distributed over both lower extremities .Histologic examination of a perilesional skin biopsy sample revealed a subepidermal blister with abundant eosinophils, neutrophil and lymphocyte. Direct immunofluorescence (IF) examination showed linear deposits of IgA along the basement membrane zone (BMZ). Diagnosis:linear IgA bullous dermatosis of childhood.