A Case of Esophageal Involvement in Pemphigus Vulgaris / 대한소화기내시경학회지

ABSTRACT

Pemphigus vulgaris is a rare chronic disorder that is characterized by the development of bullae on the skin and mucous membrane due to an acantholysis mediated by circulating autoantibodies. It begins as painful, non- healing ulcerations in the mouth. After a period of weeks to months, the condition progresses to the skin. Occasionally, skin lesions may develop as the initial manifestation of this disease. However, esophageal involvement of pemphigus vulgaris is rare. An 84-year-old man was referred due to chest pain, dysphagia, oral ulcerations and facial bullae. The histopathological findings showed a suprabasal blister and acantholysis in the epithelium of the facial skin. An endoscopic examination revealed exfoliated erosions, ulcerations and hemorrhagic bullae on the oral and esophageal mucosa. The patient was started on high dose of corticosteroids and azathioprine, but there was no clinical improvement. The use of cyclophosphamide led to the remission of the lesions.