中国麻风皮肤病杂志 ›› 2024, Vol. 40 ›› Issue (6): 416-418.doi: 10.12144/zgmfskin202406416

• 临床研究 • 上一篇    下一篇

以水疱大疱为首发症状的大疱性系统性红斑狼疮一例并文献复习

孙琳1,2,笪美红2,董正邦2,王飞2   

  1. 1东南大学医学院，江苏南京，210003；2南京东南大学附属中大医院皮肤科，江苏南京 210009
  • 出版日期:2024-06-15 发布日期:2024-05-08

Bullous systemic lupus erythematosus with blisters and bullae as the first symptom: a case report and literature review

SUN Lin1,2, DA Meihong2, DONG Zhengbang2, WANG Fei2   

  1. 1 School of Medicine, Southeast University, Nanjing 210003, China; 2 Department of Dermatology, Zhongda Hospital Southeast University, Nanjing 210009, China
  • Online:2024-06-15 Published:2024-05-08

PDF (PC)

252

摘要： 患者，女，18岁。全身红斑、水疱、糜烂1年。皮肤科检查：头颈、四肢散在浅表糜烂，躯干水肿性红斑上可见簇集性小水疱，多发浅表糜烂，双掌及手背摩擦部位多发红斑，口腔黏膜糜烂。抗核抗体（ANA）阳性，抗VII型胶原抗体阳性。皮肤组织病理示：表皮轻微角化过度，表皮下裂隙形成，真皮浅层胶原及血管周围可见大量炎细胞浸润，局部成片状。直接免疫荧光示：真表皮交界处可见线状IgG沉积;盐裂皮肤间接免疫荧光阴性。诊断：大疱性系统性红斑狼疮。

关键词: 大疱性系统性红斑狼疮, 水疱, 大疱, 自身免疫

Abstract: An 18-year-old girl presented with blisters and erythema on the whole body in one year involving oral mucosal erosion. Physical examination revealed multiple erythemas, superficial erosion, and tense blisters and bullae on the face, chest, back, and extremities, with general symptoms of anemia alopecia, and arthralgia. Antinuclear antibodies (ANA)and autoantibodies to type VII collagen were positive. Biopsy showed slight hyperkeratosis of the epidermis, subepidermal fissure formation, and a large number of inflammatory cell infiltrations in the superficial dermis and around the collagen vessels. Direct immunofluorescence showed linear deposits of IgG at the basement membrane zone, the dermis, and the epidermis, and IIF was negative via the salt-split skin technique. Eventually, bullous systemic lupus erythematosus was diagnosed.

Key words: bullous systemic lupus erythematosus, blisters, bullae, autoimmunity