Bullous Pemphigoid Beyond the Skin: A Rare Cause of Oesophageal Haemorrhage

Abstract

Background: Bullous pemphigoid (BP) is a chronic autoimmune bullous disease that predominantly affects elderly individuals with multiple comorbidities. Oesophageal involvement is uncommon, accounting for approximately 4% of cases. Oesophageal BP typically presents as chest pain, dysphagia, odynophagia or can remain entirely asymptomatic.
Case presentation: We report a 72-year-old female with a history of heart failure, atrial fibrillation on apixaban, and recently diagnosed BP on prednisone. She presented with acute hematemesis while sleeping and recurrent overnight blood-tinged expectoration. She had consumed hard nuts the previous night. She denied prior gastrointestinal bleeding or other risk factors.
Physical examination of the mouth revealed healing cutaneous BP lesions and dark red macules on the hard palate. Urgent esophagogastroduodenoscopy (EGD) revealed fragile, bullous oesophageal lesions. A tissue biopsy attempt was aborted due to immediate, excessive bleeding. Routine gastric biopsies revealed mild gastritis without malignancy or helicobacter pylori. No alternative bleeding sources were identified.
Throughout admission, the patient had no further haemorrhage or melena. Haemoglobin gradually stabilised at 11 g/dL without requiring transfusions. The hematemesis was attributed to mucosal injury of oesophageal BP lesions precipitated by mechanical trauma from hard food. Apixaban was safely resumed, and she was discharged on a high-dose proton pump inhibitor, a soft food diet, and her home maintenance BP therapy.
Conclusion: This case highlights the importance of suspecting mucosal BP in patients with upper gastrointestinal bleeding. Recognising this involvement allows for a modified procedural approach, ensuring cautious manipulation to mitigate the risk of severe, procedure-induced mucosal trauma or haemorrhage.