Linear IgA disease

Purpose: A case of linear IgA disease is reported to alert ophthalmologists and physicians to this unusual cause of chronic cicatrizing conjunctivitis . Methods: Clinical records of a patient suffering from linear IgA disease we re reviewed. Results: A 65-year-old woman with a complicated medical history experienced rapidly progressive chronic cicatrizing conjunctivitis leading to corneal perforation. Undiagnosed gingivitis and palatal ulceration had been present for 5 years prior to the onset of ocular symptoms and vitamin C deficiency had followed the consequent dietary restrictions. A diagnosis of linear Ig A disease was made on conjunctival biopsy, which demonstrated linear deposi ts of IgA along the epithelial basement membrane. The perforation was manag ed successfully with a conjunctival pediculate flap. Control of the inflamm ation was achieved with systemic prednisolone and cyclophosphamide but at t he expense of serious systemic side-effects. Conclusions: Linear IgA disease causes progressive conjunctival cicatrizati on in many affected individuals. Although dapsone generally controls the in flammation, heavier systemic immunosuppression was required in this case. I nvolvement of skin or other mucosal surfaces may become symptomatic before the conjunctivitis, and physicians must be educated to refer patients for o phthalmological review on diagnosis. Conversely, ophthalmologists encounter ing ocular linear IgA disease should be aware of the possibility of other m ucosal involvement requiring physician intervention.