Esophageal Cicatricial Pemphigoid as an Isolated Involvement Treated with Mycophenolate Mofetil

Abstract EN

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.