Abstract

Study design: Retrospective study
Purpose: To report clinical features and outcomes of ocular cicatricial pemphigoid(OCP) with systemic connective tissue disorders(CTD).
Methods: Data was reviewed from medical records of patients with OCP and CTD. Immunofluorescence of conjunctival biopsy samples was done to diagnose OCP.
Results: Ten eyes of five patients were studied. Median age was 54 years with 4:1 female to male ratio. Out of 5 patients, 4 had rheumatoid arthritis while 1 had primary Sjogren's syndrome. Forniceal shortening was seen in 6(60%)eyes and 4(40%)eyes had corneal scars. Four cases received systemic immunosuppression with no relapse or adverse effects. The fifth patient was retropositive and had repeated bilateral corneal perforations which progressed to phthisis bulbi. Final vision improved in 4/5 cases(p=0.001) with scleral contact lenses(SCL).
Conclusion: OCP can coexist with CTD and a high degree of clinical suspicion with systemic work up is required for diagnosis.