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.

Esophageal Stricture Recalcitrant to Repeated Dilation in Complex Rheumatological Disease

“…Mucous membrane pemphigoid most commonly affects the eye and oropharynx but can rarely affect the esophagus, nasopharynx, larynx, and skin. 3 Rupture of bullae can lead to scar and stricture formation. 9 , 11 Esophagitis dissecans can also be seen in bullous pemphigoid.…”

“… 9 Diagnosis is made by biopsy in both diseases and can show superficial inflammatory infiltrate containing lymphocytes, histiocytes, and eosinophils and shows linear deposits of IgG and/or C3. 3 , 4 Treatments include steroids and immunosuppression therapy. 4 Rupture of bullae can lead to scar and stricture formation as was thought to be the case in this patient despite lack of overt endoscopic findings.…”

“… 2 Esophageal involvement in autoimmune bullous diseases is rarely seen in the setting of widespread disease and is hardly ever reported as the only affected organ. 3 , 4 These patients can present with odynophagia, dysphagia, chest pain, hematemesis, or strictures. 5 We present the case of a patient who presented with dysphagia secondary to esophageal strictures refractory to multiple dilations that was found to be secondary to autoimmune bullous disease.…”

Esophageal Cicatricial Pemphigoid

“…1 Esophageal involvement is rare, mostly occurring in disseminated disease, with an incidence of 3% of patients with CP. 2 We report a case of CP with esophageal and ocular involvement treated with rituximab and esophageal dilation.…”

Structured Diagnostic Approach and Risk Assessment in Mucous Membrane Pemphigoid with Oesophageal Involvement

“…Oesophageal involvement is generally considered to be relatively rare (4-11%) (2)(3)(4)(5), but there is a lack of studies assessing its actual incidence. Oesophageal symptoms may sometimes represent the sole manifestation of MMP (6)(7)(8)(9). Dysphagia, odynophagia, pain in the oral cavity or throat, weight loss, and/or inability to ingest solid food have been described as indicative of MMP with oesophageal affection (5).…”