Esophageal Bullous Pemphigoid

Bullous pemphigoid is a rare autoimmune dermatologic disease that usually occurs in the elderly. Mucous membrane lesions occur in about 10-35% of patients and are almost always limited to the oral mucous membrane. Esophageal involvement is very rare (4% of cases) and usually presents with chest pain, dysphagia, and odynophagia, though patients are frequently asymptomatic. We report the case of newly diagnosed bullous pemphigoid in a 76-year-old man with a past medical history of dementia. He presented with cutaneous manifestations but also severe gastrointestinal bleeding due to extensive esophageal involvement. Although bullous pemphigoid is mainly a skin disease, mucous membrane lesions should not be overlooked as they are associated with an even poorer outcome. A high index of suspicion for esophageal involvement is needed as its presentation can be fatal, as with our patient.

Learning points: Bullous pemphigoid is a rare autoimmune disease that should be suspected in elderly patients with itchy cutaneous lesions.Mucous membrane lesions should always be evaluated, as they are associated with a poor prognosis, even if asymptomatic.Early diagnosis should be the main focus, as steroids, the mainstay of treatment, may not be effective in severe cases.