新生儿线状IgA大疱性皮病题录

患儿男, 生后10 d, 因皮肤红斑、水疱6 d就诊入院。皮肤科检查:全身皮肤散在或融合分布红斑, 在正常皮肤或红斑基础上可见大小不等的紧张性水疱, 部分水疱破溃、糜烂;口腔黏膜可见血疱、红色糜烂面。组织病理检查示表皮下水疱, 疱内可见中性粒细胞及少量嗜酸性粒细胞。直接免疫荧光检查显示:沿基底膜带有均质型线状IgA和颗粒状C3沉积, IgG阴性。诊断:新生儿线状IgA大疱性皮病。给予营养支持、防感染等综合治疗后, 皮肤红斑、水疱消退, 黏膜损害减轻出院。患儿出院后16个月电话随访, 一般情况良好, 皮肤黏膜皮损消退、愈合, 无新发皮疹, 生长发育正常。

Neonatal linear IgA bullous dermatosis

【Abstract】 A 10-day-old male infant presented with skin erythema and blisters for 6 days. Skin examination showed scattered or confluent erythema all over the body, tense blisters of varying sizes on the normal skin or an erythematous base, and some blisters were ulcerated and erosive; bloody bullae and erythematous erosive patches could be seen on the oral mucosa. Histopathological examination revealed subepidermal blisters, and there were some neutrophils and a few eosinophils in the blisters. Direct immunofluorescence assay showed homogeneous linear IgA and granular C3 deposits along the basement membrane zone, without IgG deposits. The diagnosis of neonatal linear IgA bullous dermatosis was confirmed. After comprehensive treatments including nutritional support and anti-infection treatment, skin erythema and blisters subsided, and the mucosal damage was attenuated. The telephone follow-up 16 months after discharge showed that the infant was in good general condition with normal growth and development, and the oral mucosal lesions had subsided and healed, without new skin lesions.