J. Dayrit, Christine Lyka Raymundo Sayson, Rial Juben Alcoreza De Leon, Esperanza Grace Regala Santi
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引用次数: 0
摘要
线性免疫球蛋白 A/免疫球蛋白 G 大疱性皮肤病(LAGBD)是一种罕见的自身免疫性大疱性皮肤病,其特征是循环中存在来自免疫球蛋白 A 和免疫球蛋白 G 的抗角质细胞细胞表面抗体。一名 26 岁的菲律宾籍男性病史为 4 年,在脑门红斑基础上出现多个瘙痒性小泡,并蔓延至面部、头皮、颈部、躯干上部和上肢。皮肤活检和直接免疫荧光提示诊断为 LAGBD。患者接受了食管胃十二指肠镜检查,发现十二指肠绒毛萎缩。小肠活检显示十二指肠绒毛萎缩,淋巴细胞占多数。他接受了两年的达帕酮治疗,病情明显好转。医生还建议他严格执行终身无麸质饮食。经过 5 年的随访,他只偶尔出现过非常轻微的复发,外用 5% 氯倍他索乳膏进行治疗。我们介绍了一例罕见的 LAGBD 患者合并乳糜泻的病例。还应检查全身受累情况,尤其是胃肠病。多塞松和无麸质饮食是该病例的主要治疗方法。
Linear immunoglobulin A/immunoglobulin G bullous dermatosis with celiac disease in a 26-year-old Filipino male
Linear immunoglobulin A/immunoglobulin G bullous dermatosis (LAGBD) is a rare form of autoimmune blistering disease characterized by circulating anti-keratinocyte cell surface antibodies from both the immunoglobulin A and immunoglobulin G. It presents as blisters, erosions, and pustules with erythematous lesions, primarily on the trunk and extremities. A 26-year-old Filipino male presented with a 4-year history of multiple pruritic vesicles on an erythematous base on the mentum spreading on the face, scalp, neck, upper trunk, and upper extremities. Skin biopsy and direct immunofluorescence suggest a diagnosis of LAGBD. The patient underwent esophagogastroduodenoscopy, which revealed duodenal villous atrophy. A small bowel biopsy revealed duodenal villous atrophy with a predominance of lymphocytes. He was treated with dapsone for two years with remarkable improvement. He was also advised to do a strict, lifelong gluten-free diet. After a 5-year follow-up, he only experienced occasional very mild flares treated with topical clobetasol 5% cream. We present a rare case of a patient with LAGBD with celiac disease. Systemic involvement, particularly gastroenteropathy, should also be investigated. Dapsone and a gluten-free diet have been the primary treatment modalities for this case.