Oesophageal involvement in 26 consecutive patients with mucous membrane pemphigoid. 2017

O Zehou, and J-J Raynaud, and C Le Roux-Villet, and M Alexandre, and G Airinei, and F Pascal, and M Heller, and N Lièvre, and L Laroche, and F Caux, and R Benamouzig, and C Prost-Squarcioni
Department of Dermatology and Referral Center for Auto-Immune Bullous Diseases, Avicenne Hospital, APHP, University Paris 13, 93009, Bobigny, France.

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

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009059 Mouth Diseases Diseases involving the MOUTH. Disease, Mouth,Diseases, Mouth,Mouth Disease
D010390 Pemphigoid, Benign Mucous Membrane A chronic blistering disease with predilection for mucous membranes and less frequently the skin, and with a tendency to scarring. It is sometimes called ocular pemphigoid because of conjunctival mucous membrane involvement. Pemphigoid, Cicatricial,Benign Mucosal Pemphigoid,Benign Mucous Membrane Pemphigoid,Cicatricial Pemphigoid, Ocular,Mucous Membrane Pemphigoid, Benign,Ocular Cicatricial Pemphigoid,Pemphigoid, Ocular Cicatricial,Benign Mucosal Pemphigoids,Cicatricial Pemphigoid,Cicatricial Pemphigoids, Ocular,Mucosal Pemphigoid, Benign,Mucosal Pemphigoids, Benign,Ocular Cicatricial Pemphigoids,Pemphigoid, Benign Mucosal,Pemphigoids, Benign Mucosal,Pemphigoids, Ocular Cicatricial
D003680 Deglutition Disorders Difficulty in SWALLOWING which may result from neuromuscular disorder or mechanical obstruction. Dysphagia is classified into two distinct types: oropharyngeal dysphagia due to malfunction of the PHARYNX and UPPER ESOPHAGEAL SPHINCTER; and esophageal dysphagia due to malfunction of the ESOPHAGUS. Dysphagia,Swallowing Disorders,Esophageal Dysphagia,Oropharyngeal Dysphagia,Deglutition Disorder,Disorders, Deglutition,Dysphagia, Esophageal,Dysphagia, Oropharyngeal,Swallowing Disorder
D004106 Dilatation The act of dilating. Dilation,Dilatations,Dilations
D004935 Esophageal Diseases Pathological processes in the ESOPHAGUS. Disease, Esophageal,Diseases, Esophageal,Esophageal Disease
D004945 Esophagoscopy Endoscopic examination, therapy or surgery of the esophagus. Esophagoscopic Surgical Procedures,Surgical Procedures, Esophagoscopic,Esophagoscopic Surgery,Surgery, Esophagoscopic,Esophagoscopic Surgeries,Esophagoscopic Surgical Procedure,Esophagoscopies,Procedure, Esophagoscopic Surgical,Procedures, Esophagoscopic Surgical,Surgeries, Esophagoscopic,Surgical Procedure, Esophagoscopic
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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