title: Esophageal Cicatricial Pemphigoid as an Isolated Involvement Treated with Mycophenolate Mofetil.
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.
authors.affiliation: Gastroenterology Department, Hospital Infanta Cristina, Avenida 9 de Junio, No. 2, 28981 Parla, Madrid, Spain.
authors.forename: Sandra
authors.initials: S
authors.lastname: Sánchez Prudencio
authors.affiliation: Gastroenterology Department, Hospital Infanta Cristina, Avenida 9 de Junio, No. 2, 28981 Parla, Madrid, Spain.
authors.forename: Daniel
authors.initials: D
authors.lastname: Domingo Senra
authors.affiliation: Gastroenterology Department, Hospital Infanta Cristina, Avenida 9 de Junio, No. 2, 28981 Parla, Madrid, Spain.
authors.forename: Daniel
authors.initials: D
authors.lastname: Martín Rodríguez
authors.affiliation: Gastroenterology Department, Hospital Infanta Cristina, Avenida 9 de Junio, No. 2, 28981 Parla, Madrid, Spain.
authors.forename: Belén
authors.initials: B
authors.lastname: Botella Mateu
authors.affiliation: Gastroenterology Department, Hospital Infanta Cristina, Avenida 9 de Junio, No. 2, 28981 Parla, Madrid, Spain.
authors.forename: Carlos
authors.initials: C
authors.lastname: Esteban Jiménez-Zarza
authors.affiliation: Gastroenterology Department, Hospital Infanta Cristina, Avenida 9 de Junio, No. 2, 28981 Parla, Madrid, Spain.
authors.forename: Felipe
authors.initials: F
authors.lastname: de la Morena López
authors.affiliation: Dermatology Department, Hospital Infanta Cristina, Avenida 9 de Junio, No. 2, 28981 Parla, Madrid, Spain.
authors.forename: José
authors.initials: J
authors.lastname: Jiménez Reyes
authors.affiliation: Pathology Department, Hospital Infanta Cristina, Avenida 9 de Junio, No. 2, 28981 Parla, Madrid, Spain.
authors.forename: Manuel
authors.initials: M
authors.lastname: Nevado Santos
authors.affiliation: Gastroenterology Department, Hospital Infanta Cristina, Avenida 9 de Junio, No. 2, 28981 Parla, Madrid, Spain.
authors.forename: Beatriz
authors.initials: B
authors.lastname: de Cuenca Morón