Esophageal Cicatricial Pemphigoid as an Isolated Involvement Treated with Mycophenolate Mofetil

• Published in: Case reports in gastrointestinal medicine 2015-01, Vol.2015 (2015), p.1-4
• Main Authors: de Cuenca Morón, Beatriz, Nevado Santos, Manuel, de la Morena López, Felipe, Esteban Jiménez-Zarza, Carlos, Botella Mateu, Belén, Martín Rodríguez, Daniel, Domingo Senra, Daniel, Sánchez Prudencio, Sandra, Jiménez Reyes, José
• Format: Article
• Language: English
• Subjects: Case Report
• ISSN: 2090-6528; 2090-6536
• DOI: 10.1155/2015/620374

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.