大疱性系统性红斑狼疮1例

Bullous Systemic Lupus Erythematosus Misdiagnosed as Pemphigus: Case Report

患者女,32岁,躯干红斑、水疱伴瘙痒40 d,口腔黏膜糜烂半个月。皮肤科情况:双侧腋下、躯干散在暗红色斑片,双侧腋下可见散在松弛性水疱,尼氏征阴性,口腔黏膜部分糜烂。抗核抗体(ANA)、抗PO抗体、抗SmD1抗体、U1-snRNP抗体均阳性,补体C3、C4降低。皮损组织病理:表皮下水疱形成,疱液内见淋巴细胞、中性粒细胞浸润,表皮浅层及血管周见中等量淋巴细胞、中性粒细胞浸润。直接免疫荧光(DIF):IgA、IgM基底膜带处线状沉积(++),IgG基底膜带处线状沉积(±),C3阴性。诊断:大疱性系统性红斑狼疮。给予糖皮质激素及硫酸羟氯喹治疗后效果不佳,加用氨苯砜后效果显著。

A 32-year-old female patient presented with erythema,blister and pruritus on the trunk for 40 days and erosion of oral mucosa for half a month.The skin lesions showed dark red patches in the bilateral axilla and trunk,scattered flaccid blisters in the bilateral axilla,negative nissner’s sign,and partial erosion of oral mucosa.ANA,anti-PO,anti-SMD1 and U1-SNRNP antibodies were all positive,and complement C3 and C4 decreased.Skin lesion histopathology showed blister formation under the epidermis,infiltration of lymphocytes and neutrophils in the blister fluid,moderate amount of lymphocytes and neutrophils in the superficial epidermis and perivascular layer.Direct immunofluorescence(DIF):linear deposition in IgA and IgM basement membrane bands(++),linear deposition in IgG basement membrane bands(±),C3 negative.Diagnosis:bullous systemic lupus erythematosus.The effect of glucocorticoid and hydroxychloroquine sulfate was not good,but the effect of dapsone was significant.