大疱性系统性红斑狼疮1例

A Case of Bullous Systemic Lupus Erythematosus

患者女,22岁。躯干、四肢出现皮疹1个月,水疱半个月。皮损组织病理示:表皮下水疱,伴有大量淋巴细胞、中性粒细胞浸润,真皮浅层血管及附件周围大量淋巴细胞、中性粒细胞浸润。大疱性类天疱疮自身抗体Bp180抗体检测(-)。完善抗核抗体谱及直接免疫荧光检查,ANA:颗粒型(+),胞浆型1∶320;Sm:(+);SS-A(天然60k Da):(+);SS-A(重组Ro52k Da):(+)。直接免疫荧光结果:Ig A,Ig M,C3基底膜荧光(+)。诊断:大疱性系统性红斑狼疮。

A 22-year-old female presented with lesions on truck and limbs for 1 month. Histopathological finding showed formation of subepidermal blister with large numbers of lymphocytes and neutrophils, and some lymphocytes and neutrophils around vessles and the skin appendages in the superficial plexus. Her serum antibody Bpl80 was negative. Her speckled type and peripheral type （ titer 1 ： 320） antinuclear antibodies, anti-Sm, anti-SSA （natural 60kDa）, anti-SSA （Restructuring Ro52kDa） antibodies were positive. A direct immunofluorescence study showed deposition of IgA, IgM and C3 at the basement membrane zone. A diagnose of bullous systemic luous ervthematosus was made.