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Case Reports in Gastrointestinal Medicine
Volume 2015, Article ID 620374, 4 pages
http://dx.doi.org/10.1155/2015/620374
Case Report

Esophageal Cicatricial Pemphigoid as an Isolated Involvement Treated with Mycophenolate Mofetil

1Gastroenterology Department, Hospital Infanta Cristina, Avenida 9 de Junio, No. 2, 28981 Parla, Madrid, Spain
2Dermatology Department, Hospital Infanta Cristina, Avenida 9 de Junio, No. 2, 28981 Parla, Madrid, Spain
3Pathology Department, Hospital Infanta Cristina, Avenida 9 de Junio, No. 2, 28981 Parla, Madrid, Spain

Received 17 August 2015; Accepted 27 September 2015

Academic Editor: Haruhiko Sugimura

Copyright © 2015 Sandra Sánchez Prudencio et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Cicatricial pemphigoid (CP) is a rare blistering autoimmune disease. Esophageal involvement occurs in widespread disease and rarely appears as the only affected organ. We report a 67-year-old Caucasian female with esophageal dysphagia and weight loss. Several oral panendoscopies showed multiple exudative ulcerations with fibrin and webs in mid- and proximal esophagus and a peeling mucosa. There were no lesions in other organs. We established the diagnosis performing a direct immunofluorescence (DIF), demonstrating IgG3 and complement deposition along the basement membrane. As initial treatment the patient received prednisone 60 mg and 1 gr twice daily of mycophenolate mofetil (MMF) as a steroid-sparing agent due to its lower toxicity and its selective mechanism of action. Six months later there was a significant clinical improvement and the esophageal ulcerations had disappeared, developing cicatricial fibrous rings, although no stenosis was present. Four years later, the patient remains asymptomatic with a low maintenance dose of MMF.