A Rare Case of Linear IgA Bullous Disease With Oesophageal Involvement.

Objectives
Linear IgA bullous disease (LABD) is a rare autoimmune blistering disorder characterised by linear deposition of IgA along the basement membrane zone. It typically affects the skin and oral mucosa, though other mucosal surfaces may be involved. Oesophageal manifestations are exceptionally rare and poorly represented in the literature. This case highlights the potential for oesophageal involvement in LABD and the value of multidisciplinary management in multisite disease.

Case
A 44-year-old female with LABD affecting oral and cutaneous sites is managed jointly by Oral Medicine and Dermatology. She initially presented to Bristol Dental Hospital with a sudden onset of oral blistering and ulceration. Biopsy and direct immunofluorescence confirmed LABD and she was subsequently referred to Dermatology to manage her widespread vesicular rash. She commenced a topical corticosteroid mouthwash, 100mg Doxycycline daily, and 50mg Dapsone on alternate days.

Several months later, she reported a 10-day history of high dysphagia and retrosternal discomfort to solids and liquids, with no other systemic symptoms. This was initially suspected to represent doxycycline induced oesophagitis and was treated with 40mg Omeprazole, with little improvement. Gastroscopy revealed circumferential ulceration of the oesophagus. Biopsies excluded infection, malignancy, and demonstrated a subepithelial split, supporting oesophageal involvement secondary to LABD.

Management included continuation of Dapsone with haematological monitoring and discontinuation of Doxycycline under coordinated care between Oral Medicine, Dermatology, and Gastroenterology. The patient reported gradual improvement in oesophageal symptoms, with follow-up showing resolution.

Conclusions
LABD can extend beyond cutaneous and oral sites to involve the gastrointestinal tract. In patients with upper gastrointestinal symptoms, early recognition of oesophageal involvement allows prompt treatment and reduces the risk of complications such as strictures or nutritional compromise. Multidisciplinary collaboration remains essential to ensure comprehensive management and optimal outcomes in complex mucocutaneous disease.