成人线状IgA大疱性皮病4例及文献回顾

发布时间：2018-06-07 21:28

本文选题：成人线状IgA大疱性皮病 + 　；参考：《中国皮肤性病学杂志》2017年03期

【摘要】：目的了解成人线状Ig A大疱性皮病的临床特点。方法回顾性分析本院经治的4例及既往国内文献报道的15例成人线状Ig A大疱性皮病的临床资料。结果共19例,其中男12例,女7例,平均发病年龄(39.42±17.12)岁。皮损好发于躯干及四肢,头、面、颈、口腔黏膜和会阴部位亦可受累。较之儿童型,成人皮疹更具多形性。典型皮损组织病理示:表皮下水疱或裂隙形成,真皮乳头及真皮浅层可见中性、嗜酸性粒细胞及淋巴细胞等炎症细胞浸润。直接免疫荧光见基底膜带Ig A线状沉积,可伴C_3沉积,部分伴Ig G和Ig M沉积。氨苯砜、糖皮质激素及多种免疫抑制剂等治疗效果较好。结论本病好发于20~50岁人群,病程慢性,部分可自行缓解,诊断依赖组织病理及直接免疫荧光检查,治疗首选氨苯砜。
[Abstract]:Objective to investigate the clinical features of linear Ig A bullous dermatosis in adults. Methods the clinical data of 4 cases treated by our hospital and 15 cases of adult linear Ig A bullous dermatosis were retrospectively analyzed. Results there were 19 cases, including 12 males and 7 females, with an average age of 39.42 卤17.12 years. Lesions may occur in the trunk and limbs, head, face, neck, oral mucosa and perineum. Adult rashes are more pleomorphic than children's. Histopathology of typical lesions showed the formation of subepidermal blister or fissure, neutrophil, eosinophilic granulocyte and lymphocyte infiltration in dermis papilla and superficial dermis. Direct immunofluorescence was observed in the basal membrane zone with Ig A linear deposition, which could be accompanied by CST3 deposition, and partly with Ig G and IgM deposition. Dapsone, glucocorticoid and various immunosuppressants are effective. Conclusion the disease is prone to occur in 2050 years old people, the course of disease is chronic, partial self-remission, the diagnosis depends on histopathology and direct immunofluorescence examination, the first choice of treatment is dapsone.
【作者单位】：郑州大学临床医学系;
【分类号】：R758.66

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