CORNEAL PERFORATION IN THE CONTEXT OF OCULAR CICATRICIAL PEMPHIGOID

This case report aims to highlight the clinical signs of OCP and discuss its management and potential complications to enhance early diagnosis and treatment.

Cicatricial pemphigoid is a rare autoimmune blistering disorder primarily affecting mucosal membranes and, less commonly, the skin. Clinically, it manifests with erythematous plaques, blisters, and erosions that heal with scarring.Ocular cicatricial pemphigoid (OCP) is an even rarer form, with an incidence of 0.8 per million. OCP features conjunctival inflammation, subepithelial blistering, and progressive scarring, leading to severe complications like trichiasis, entropion, and corneal perforation. Early diagnosis and prompt immunosuppressive therapy are essential to prevent irreversible damage

This is the case of a 70-year-old woman who had experienced ocular symptoms such as a burning sensation, foreign body sensation, tearing, and occasional pain for many years. She also reported oral discomfort and some skin lesions. Due to suspected cicatricial pemphigoid with ocular involvement, mucosal biopsies and a complete autoantibody profile were performed. The biopsy showed subepithelial lymphoplasmacytic infiltration in the conjunctival and oral mucosa, with positive ANA and Anti-Ro autoantibodies, suggesting associated Sjögren’s syndrome. Treatment was initiated with mycophenolate but switched to dapsone and cyclophosphamide due to poor tolerance. During this period, the ophthalmological examination revealed eyelid thickening, fornix synechiae, reduced tear break-up time, meibomitis, superficial punctate keratitis, and inferior symblepharon. These conditions were treated with intensive ocular lubrication, insulin eye drops, dexamethasone, and topical cyclosporine.

Two months after starting topical cyclosporine, the patient presented to the ophthalmology emergency department with severe pain in the right eye. Examination revealed a 2x2 mm corneal ulcer with central microperforation and spontaneous Seidel’s sign. At that time, air was injected into the anterior chamber under aseptic conditions, a therapeutic contact lens was placed, and a compressive bandage was applied. After five days, the microperforation had closed. However, the corneal ulcer persisted one month after the resolution of the ocular perforation. Autologous serum eye drops were added to the treatment, and an amniotic membrane was implanted on the cornea. Subsequently, the corneal defect healed completely, and the patient has remained stable and symptom-free for the past six months.

OCP is a rare but severely limiting condition for patients. Close multidisciplinary monitoring is essential for effective disease management. Recognizing ophthalmological signs is crucial for early suspicion and treatment to prevent complications. In cases of corneal perforation, it is crucial to act as quickly as possible and maintain close monitoring until it is fully resolved.