Mucous membrane pemphigoid with severe esophageal stricture

Area of Science:

• Immunodermatology
• Gastroenterology
• Autoimmune blistering diseases

Background:

• Mucous membrane pemphigoid (MMP) is a rare autoimmune blistering disease primarily affecting mucous membranes, often leading to scarring.
• Esophageal involvement in MMP is uncommon, typically occurring in patients with extensive disease, presenting as membranes or strictures.

Observation:

• This case report details a patient with MMP presenting solely with severe esophageal strictures, notably lacking any cutaneous manifestations.
• The patient's condition involved significant esophageal narrowing, indicative of advanced disease progression.

Findings:

• Treatment with intravenous immunoglobulin (IVIG) led to complete remission of the severe esophageal strictures.
• The successful outcome highlights the efficacy of IVIG in managing refractory MMP with esophageal involvement.

Implications:

• This case suggests that IVIG can be a valuable therapeutic option for mucous membrane pemphigoid patients with severe esophageal strictures, even in the absence of skin lesions.
• Further research into the role of IVIG in gastrointestinal manifestations of autoimmune blistering diseases is warranted.