大疱性系统性红斑狼疮一例

A case of bullous systemic lupus erythematosus

65岁女性患者,全身红斑、水疱伴瘙痒1个月。面部、躯干、四肢散在分布暗红斑,红斑基础上可见松弛性水疱或大疱,尼氏征阴性,口腔颊黏膜散在浅表糜烂。左肩部新发红斑处皮损组织病理:表皮下裂隙、水疱形成,疱内少量嗜酸粒细胞及真皮浅层淋巴细胞、中性粒细胞浸润。直接免疫荧光可见Ig A、Ig G和补体C3在表皮下呈线状沉积。间接免疫荧光显示抗基膜带抗体阳性(滴度1:80)。左面颊皮损组织病理:表皮萎缩变薄,表真皮分离,真皮浅中层血管扩张,周围散在淋巴细胞为主的炎性细胞浸润,胶原嗜碱变性。抗核抗体(ANA)阳性(1:320);Sm抗体阳性;动态红细胞沉降率60 mm/h;补体C3 0.59 g/L,C1q 129 mg/L。诊断:大疱性系统性红斑狼疮。经甲泼尼龙联合硫酸羟氯喹治疗2周后好转出院,随访6个月无复发。

A 65-year-old female patient presented with erythema, blister over the whole body with itching for 1 month. Physical examination: dark red spots on the face, trunk and limbs, as well as loose blister and bullae, Nikolsky sign negative, superficial erosion on the oral mucosa. Histopathology of the lesions:(left shoulder) there were cracks and vesicles under the epidermis, and a small amount of eosinophils, lymphocytes and neutrophils infiltrated in the superficial dermis;direct immunofluorescence: Ig A/Ig G/C3: linear deposition under the epidermis;indirect immunofluorescence: anti-basement membrane band antibody: 1:80;(left cheek) the epidermis was atrophic and thinned, superficial dermis separation, the vessels in the superficial and middle dermis were dilated, the inflammatory cells mainly composed of lymphocytes scattered around, and the collagen was alkalophilic. ANA positive:1:320;Sm:positive;ESR: 60 mm/h;C3:0.59 g/L;C1 q:129 mg/L. The final diagnosis: bullous systemic lupus erythematosus. After treatment with methylprednisolone and hydroxychloroquine sulfate for 2 weeks, the patient was discharged from hospital with no recurrence after follow-up for 6 months.