F1000Research (Jan 2022)
A case series of ocular involvement in bullous pemphigoid: clinical features, management, and outcomes [version 2; peer review: 2 approved]
Abstract
Ocular involvement in cases of bullous pemphigoid is rare and when present, the signs are usually subtle and in the form of fine tarsal scarring and dry eye disease. The current report aims to describe the clinical features and management protocols in a series of cases with aggressive ocular manifestations at presentation. All cases of bullous pemphigoid seen between 2017 and 2020 were included in this retrospective case series. Data regarding the clinical features, treatment administered, and outcomes was collected. Five cases (n=10 eyes) of bullous pemphigoid disease with ocular involvement were included. All eyes had significant cicatricial conjunctival changes in the form of symblephara, inferior forniceal shortening, and tarsal conjunctival scarring. Conjunctival granulomas were present in 3/10 eyes. Corneal involvement in the form of punctate keratitis was present in all eyes while 4/10 eyes had an epithelial defect as well. The management of these cases involved topical therapy with corticosteroids and lubricants (n=10 eyes) while pulse doses of intravenous methyl prednisolone were administered in 5/5 cases. Pulse intravenous cyclophosphamide was supplemented in 2/5 cases. Adequate control of the disease was noted in 3/5 cases while one case had a recalcitrant form of the disease and developed a dermalised ocular surface in both eyes. The last patient was lost to follow up during the course of therapy. Bullous pemphigoid can present with an aggressive form of cicatrizing conjunctivitis similar to other variants of autoimmune blistering disorders and must be considered as a differential in cases presenting with ocular cicatricial disease. Long-term intensive immunosuppression is required for the management of these cases to preserve the visual function and the integrity of the globe.
