Progression and management Linear IgA disease with oral, pharyngeal and ocular involvement: A Case Report

Background
Linear IgA disease(1) is a rare, auto-immune mucocutaneous blistering condition, which can be difficult to diagnose and manage(2). This case describes the progression of a well-localised, asymptomatic area of desquamative gingivitis to multi-site linear IgA disease with oropharyngeal and ocular involvement, eventually requiring systemic therapy and surgical intervention.

Case Summary
A 76-year-old man was referred to Oral Medicine by his dentist regarding an asymptomatic area of suspected desquamative gingivitis confined to the lower anterior gingivae. Medically he had longstanding Rheumatoid Arthritis and was taking sulfasalazine. Establishing this patient’s oral diagnosis was complicated by the insidious onset of the disease. Initial biopsy results showed non-specific inflammation and ulceration, and clear histopathological evidence, supported by immunofluorescence studies, was only available following disease progression to a multi-site blistering appearance. Medical management of this patient was complicated by the patient’s reluctance to consider systemic therapies, on the basis of concerns around side effects, and worries about potential impact on his Rheumatoid Arthritis treatment. Further complications included inefficacy of topical therapies for oropharyngeal symptoms, contraindication of dapsone due to chronically low haemoglobin, and failure of two other therapy trials. Further progression of the disease necessitated ocular surgery to manage the disease complications.

Conclusion
This report highlights the presentation of a developing case of linear IgA disease, and suggests therapeutic options for the management of this disease at various levels of severity, along with useful reflections on patient-centred decision making when escalating treatment.

1. Shin L, Gardner JT 2nd, Dao H Jr. Updates in the Diagnosis and Management of Linear IgA Disease: A Systematic Review. Medicina (Kaunas). 2021 Aug 12;57(8):818. doi: 10.3390/medicina57080818. PMID: 34441024; PMCID: PMC8400507.
2. Khan M, Park L, Skopit S. Management Options for Linear Immunoglobulin A (IgA) Bullous Dermatosis: A Literature Review. Cureus. 2023 Mar 21;15(3):e36481. doi: 10.7759/cureus.36481. PMID: 37090290; PMCID: PMC10115698.