Department of Gastroenterology, Centro Hospitalar de Leiria, Leiria, Portugal.
Acta Gastroenterol Belg. 2021 Apr-Jun;84(2):365-366. doi: 10.51821/84.2.365.
Pemphigus vulgaris (PV) is a rare autoimmune blistering disorder of the skin and mucous membranes. The true prevalence of esophageal involvement is unknown; esophageal symptoms almost always occur in the context of oral mucosa involvement. We report the case of a 66-year-old man with cutaneous blisters and esophageal symptoms that did not respond to acid suppression therapy. Esophagogastroduodenoscopy showed esophageal ulcers and mucosal desquamation. Biopsies were consistent with the diagnosis of PV. The patient was started on immunosuppressive therapy, achieving remission. This represents a rare case of esophageal involvement of PV without mucosal involvement and draws attention to a rare cause of dysphagia, which can be fatal if left untreated.
寻常性天疱疮(PV)是一种罕见的皮肤黏膜自身免疫性水疱病。食管受累的真实患病率尚不清楚;食管症状几乎总是在口腔黏膜受累的背景下出现。我们报告了一例 66 岁男性,其皮肤出现水疱和食管症状,这些症状对抑酸治疗没有反应。食管胃十二指肠镜检查显示食管溃疡和黏膜脱落。活检结果与 PV 的诊断一致。患者开始接受免疫抑制治疗,病情缓解。这代表了一例罕见的无黏膜受累的 PV 食管受累病例,并提醒人们注意一种罕见的吞咽困难病因,如果不治疗,可能会致命。