Corneal Surgery In Ocular Cicatricial Pemphigoid: A Multidisciplinary Approach For Managing Inflammation

Ocular cicatricial pemphigoid (OCP) is a chronic, immune-mediated, bullous, cicatricial disease within the spectrum of mucocutaneous membranous pemphigoid. It encompasses severe dry‑eye syndrome, corneal erosions, ulcers and keratinization, entropion and symblepharon. The low prevalence, estimated between 1/10.000 – 1/60.000, together with the aggressive course of the disease with progression to blindness (in 20% of cases) and the need for systemic immunosuppression poses a clinical challenge even for the corneal specialist. Besides medical management, surgery can involve specialized techiques like tectonic penetrating keratoplasty (TPK) or large grafts. 

Case report of the diagnosis and management of OCP, particularly TPK. The patient was followed at the ophthalmology department of Egas Moniz Hospital (Lisbon, Portugal). The anterior segment was assessed with Haag-Streit BQ 900® LED + IM 900® slit-lamp and surgeries were recorded with LEICA® and ZEISS® surgical microscopes. 

A 72-year-old man presented with red eye and foreign body sensation. He had a history of phthisis bulbi in the right eye (RE) from a retinal detachment (RD) in infancy, cataract surgery of the left eye (LE) and glaucoma. Visual acuity (VA) was no light perception (LP) in the RE and 20/50 in the LE. Bilateral trichiasis, fornix shortening and symblepharon, RE corneal opacification and LE conjunctival hyperemia with diffuse keratitis were observed. At the time, diagnosis of pseudopemphigoid by glaucoma medication was assumed. He was submitted to cilia photocoagulation and prescribed oral acetazolamide, lubrification and topical hydrocortisone. There were repeated visits (>10/year) to the emergency room for the same superficial ocular complaints. The recurring course of cicatricial keratoconjunctivitis raised suspicion of OCP, because it did not improve with discontinuation of the offending agents. Conjunctival biopsy with immunofluorescence was negative. Corneal disease worsened, with a central ulcer, descemetocele and perforation of the LE, despite oral and topical antibiotics/steroids and topical tacrolimus/insulin. Cyanoacrylate glue, contact lens and amniotic membrane were placed, proven insufficient. A TPK was performed, repeated 3 times. A choroidal effusion developed, with subsequent RD, managed with posterior vitrectomy, retinotomy and silicone oil tamponade. Simultaneously, rituximab was started. VA waved between counting fingers and LP. 

OCP is a devastating autoimmune condition requiring a joint approach between the corneal specialist and the rheumatologist, to slow down its recalcitrant nature. Our case demonstrates the delay in diagnosis that frequently accompanies these cases (mistaken by pseudopemphigoid) and the aggressive surgical strategy that was employed to save the patient’s only eye. With immunosuppressive treatments, remission of ocular disease may be achieved, together with regular monitoring and prompt surgery for corneal (eye) preservation.