食管大疱性类天疱疮

Esophageal Bullous Pemphigoid.

作者信息

Castelo Branco Catarina, Fonseca Tomás, Marcos-Pinto Ricardo

机构信息

Department of Internal Medicine, Centro Hospitalar Universitário do Porto, Porto, Portugal.

Department of Gastroenterology, Centro Hospitalar Universitário do Porto, Porto, Portugal.

出版信息

Eur J Case Rep Intern Med. 2022 Feb 1;9(2):003160. doi: 10.12890/2022_003160. eCollection 2022.

Abstract

UNLABELLED

Bullous pemphigoid is a rare autoimmune dermatologic disease that usually occurs in the elderly. Mucous membrane lesions occur in about 10-35% of patients and are almost always limited to the oral mucous membrane. Esophageal involvement is very rare (4% of cases) and usually presents with chest pain, dysphagia, and odynophagia, though patients are frequently asymptomatic. We report the case of newly diagnosed bullous pemphigoid in a 76-year-old man with a past medical history of dementia. He presented with cutaneous manifestations but also severe gastrointestinal bleeding due to extensive esophageal involvement. Although bullous pemphigoid is mainly a skin disease, mucous membrane lesions should not be overlooked as they are associated with an even poorer outcome. A high index of suspicion for esophageal involvement is needed as its presentation can be fatal, as with our patient.

LEARNING POINTS

Bullous pemphigoid is a rare autoimmune disease that should be suspected in elderly patients with itchy cutaneous lesions.Mucous membrane lesions should always be evaluated, as they are associated with a poor prognosis, even if asymptomatic.Early diagnosis should be the main focus, as steroids, the mainstay of treatment, may not be effective in severe cases.

摘要

未标注

大疱性类天疱疮是一种罕见的自身免疫性皮肤病,通常发生于老年人。约10% - 35%的患者会出现黏膜病变,且几乎均局限于口腔黏膜。食管受累情况非常罕见(4%的病例),通常表现为胸痛、吞咽困难和吞咽痛,不过患者常常没有症状。我们报告一例76岁男性新诊断的大疱性类天疱疮病例,该患者有痴呆病史。他既有皮肤表现,还因广泛的食管受累出现严重的胃肠道出血。虽然大疱性类天疱疮主要是一种皮肤病,但黏膜病变不应被忽视,因为它们与更差的预后相关。需要高度怀疑食管受累情况,因为其表现可能是致命的,就像我们的患者一样。

学习要点

大疱性类天疱疮是一种罕见的自身免疫性疾病,对于有皮肤瘙痒病变的老年患者应怀疑此病。应始终评估黏膜病变,因为它们与预后不良相关,即使没有症状。早期诊断应是主要关注点,因为作为主要治疗手段的类固醇在重症病例中可能无效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/241e/8900567/3ededc7ab871/3160_Fig1.jpg

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