以环状红斑为主要表现的大疱性系统性红斑狼疮1例

A Case of Bullous Systemic Lupus Erythematosus Presented with Annular Erythema as the Main Manifestaion

患者女,12岁,全身皮肤出现红斑、水疱半年。皮肤科情况:躯干、四肢可见弥漫性分布的、大小不等的环形红斑,大部分呈同心圆状,边缘散在少许水疱,无瘢痕形成。血常规示白细胞计数:3.46×10^(9)/L,补体C348.16 mg/dL,补体C47.25mg/d L,ANA、抗ds-DNA抗体、抗Sm抗体、抗nRNP抗体、抗SSA抗体、抗SSB抗体、抗Ro-52抗体均阳性。皮损组织病理示:表皮下水疱形成,浅层周围血管淋巴细胞、中性粒细胞浸润。直接免疫荧光:C3真表皮交界处颗粒状、线状沉积。IgG、IgA、IgM真表皮交界处线状沉积。诊断:大疱性系统性红斑狼疮。治疗:给予甲泼尼龙30 mg/d、巴瑞替尼2 mg/d治疗,1周后皮损基本消退,遗留色素沉着斑。随访4个月,未见复发。

A 12-year-old girl presented with erythema and blister over the entire body with a history of half year.On physical examination,she had a lot of diffuse distribution annular erythema over the torso and limbs,most of which presented with concentric round erythema,with vesicles on the periphery.No scar was observed.Laboratory tests showed leukopenia(3.46×10^(9)/L),and decreased levels of C3(48.16 mg/dL),C4(7.25 mg/dL).Autoimmune antibodies including Antinuclear antibodies(ANA),anti-dsDNA antibodies,anti-Sm antibodies,anti-nuclear ribonucleoproteins(nRNP),anti-SSA antibodies,anti-SSB antibodies,anti-Ro-52 antibodies were positive.The histopathology of the lesion showed subepidermal bullae with inflammatory cell infiltratation of lymphocytes and neutrophils round the peripheral vascular.Direct immunefluorescence showed that granular,linear deposits of C3 and linear deposits of IgG,IgA,IgM at the basement membrane zone(BMZ).The diagnosis of bullous systemic lupus erythematosus was confirmed.The patient was administrated with Methylprednisolone 30mg/day and baritinib 2mg/day for 1 week,resulting in skin lesions basically disappeared and hyperpigmentation remained.No recurrence observed after 4 months follow-up.