大疱性系统性红斑狼疮二例

Bullous systemic lupus erythematosus:two cases report

2例患者均为女性,年龄分别为36岁和19岁。临床表现均为红斑基础上的水疱,尼氏征阴性。皮肤组织病理检查示:表皮角化不全,有中性粒细胞浸润,基底细胞液化变性。直接免疫荧光示IgA、lgG和C3呈线状或带状沉积于基底膜带。临床表现及实验室检查均符合系统性红斑狼疮的诊断标准。均给予糖皮质激素治疗后好转。

The two patients were females,and the age was 36 and 19 years old respectively.Both patients presented with blisters on the erythema with negative Nikolsky sign.Histopathological examination of the skin showed epidermal parakeratosis,neutrophil infiltration,and liquefaction degeneration of basal cells.Direct immunofluorescence showed that IgA,lgG,and C3 were deposited linearly or zonally on the basement membrane zone.The diagnosis of bullous systemic lupus erythematosus was made according the clinical manifestations and laboratory tests.Both were treated with glucocorticoids and their lesions were improved.