Case Reports in Gastrointestinal Medicine (Jan 2015)

Esophageal Cicatricial Pemphigoid as an Isolated Involvement Treated with Mycophenolate Mofetil

  • Sandra Sánchez Prudencio,
  • Daniel Domingo Senra,
  • Daniel Martín Rodríguez,
  • Belén Botella Mateu,
  • Carlos Esteban Jiménez-Zarza,
  • Felipe de la Morena López,
  • José Jiménez Reyes,
  • Manuel Nevado Santos,
  • Beatriz de Cuenca Morón

DOI
https://doi.org/10.1155/2015/620374
Journal volume & issue
Vol. 2015

Abstract

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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.