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26 例黏膜类天疱疮患者的食管受累情况。

Oesophageal involvement in 26 consecutive patients with mucous membrane pemphigoid.

机构信息

Department of Dermatology and Referral Center for Auto-Immune Bullous Diseases, Avicenne Hospital, APHP, University Paris 13, 93009, Bobigny, France.

Department of Gastroenterology, Avicenne Hospital, APHP, University Paris 13, 93009, Bobigny, France.

出版信息

Br J Dermatol. 2017 Oct;177(4):1074-1085. doi: 10.1111/bjd.15592. Epub 2017 Oct 1.

Abstract

BACKGROUND

Oesophageal involvement of mucous membrane pemphigoid (MMP) has not yet been thoroughly described.

OBJECTIVES

To characterize systematically the endoscopic lesions of a series of patients with oesophageal symptoms seen at a referral centre for autoimmune bullous diseases.

METHODS

Clinical, endoscopic and immunological findings of consecutively referred patients with MMP with oesophageal involvement, systemic and endoscopic treatments, and follow-up are described.

RESULTS

Of 477 consecutive patients with MMP consulting between 2002 and 2012, 26 (5·4%) had symptomatic oesophageal involvement. Dysphagia, observed in 23 (88%) patients, was the most frequent symptom. Oesophageal symptoms could be the first sign of MMP. Patients with oesophageal involvement had a mean of three other involved sites. At initial oesophageal endoscopy, 17 of 26 patients had active lesions (intact bullae, erosions and/or erythema), 15 had stricture(s) and 12 had other cicatricial lesions. Systemic therapy alone achieved oesophageal symptom relief for five patients. Dilatation was combined with systemic therapy for 12 patients and was successful in nine; one perforation occurred.

CONCLUSIONS

Symptomatic oesophageal involvement affected 5·4% of patients with MMP. Dermatologists and gastroenterologists should be aware of these mucocutaneous diseases and their oesophageal involvement, as it could lead to earlier diagnosis and better care. Oesophageal dilatation could be a therapeutic option for symptomatic stricture not relieved by optimized systemic therapy alone.

摘要

背景

黏膜性类天疱疮(MMP)的食管受累尚未得到充分描述。

目的

系统描述一系列在自身免疫性大疱性疾病转诊中心就诊的有食管症状的患者的内镜下病变。

方法

描述了连续转诊的 MMP 伴食管受累、系统和内镜治疗以及随访的患者的临床、内镜和免疫学发现。

结果

在 2002 年至 2012 年间连续就诊的 477 例 MMP 患者中,有 26 例(5.4%)有症状性食管受累。23 例(88%)患者观察到的吞咽困难是最常见的症状。食管症状可能是 MMP 的首发症状。有食管受累的患者平均还有另外三个受累部位。在初始食管内镜检查时,26 例患者中有 17 例有活动性病变(完整大疱、糜烂和/或红斑),15 例有狭窄,12 例有其他瘢痕性病变。单独的系统治疗使 5 例患者的食管症状得到缓解。扩张术与系统治疗联合用于 12 例患者,其中 9 例成功;1 例发生穿孔。

结论

有症状的食管受累影响了 5.4%的 MMP 患者。皮肤科医生和胃肠病医生应该意识到这些黏膜皮肤疾病及其食管受累,因为这可能导致更早的诊断和更好的治疗。对于单纯优化系统治疗不能缓解的有症状的狭窄,食管扩张术可能是一种治疗选择。

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