Abstract
Purpose :
To describe the characteristics of patients affected by ocular cicatricial pemphigoid, a rare and potentially blinding disorder.
Methods :
A retrospective review of the medical records of patients with biopsy-proven ocular cicatricial pemphigoid (OCP) at Manhattan Eye Ear, Throat Hospital was performed. This review includes patients at different stages of OCP seen from January 2017 to July 2021. Age of onset, presenting symptoms and signs, presence or absence of limbal stem cell deficiency, need for immunosuppressive therapy, disease course, and visual outcome were among the data collected.
Results :
A total of 29 patient charts were analyzed. 72% of patients were female. Symblepharon and trichiasis were present at the initial visit to the tertiary care center in 82.8% and 82.8% respectively. Significant conjunctival inflammation was a presenting sign in 48.3% of patients, while 44.8% of patients presented with either symblepharon or trichiasis (or both) in an otherwise quiet non-inflamed eye. 20.7% had extraocular involvement, of which oral lesions were the most common. 72.4% of patients required immunomodulatory therapy, and almost one fourth (23.8%) still went completely blind. 82.8% of the cohort had functional vision measured at a single time point, while 48.3% at the same time point had blindness in at least one eye.
Conclusions :
Ocular cicatricial pemphigoid is a severe ocular inflammatory condition in which patients have a high predilection of blindness even in the face of aggressive treatment.
This abstract was presented at the 2022 ARVO Annual Meeting, held in Denver, CO, May 1-4, 2022, and virtually.