Corneal perforation in ocular cicatricial pemphigoid: A CARE-compliant case report

RATIONALE: Ocular cicatricial pemphigoid (OCP) is a potentially blinding, rare systemic autoimmune disease. The definite etiology of OCP remains under debate, early diagnosis is important to prevent rapid deterioration. The majority of the discussion has been focused on its medical therapeutic strategy, while little effort has been made to study the role of amniotic membrane transplantation (AMT). We describe the first case of OCP with minimal immunosuppressant and initial ocular surface reconstruction procedure using double layer AMT. PATIENT CONCERNS: A 66-year-old female patient presented to our outpatient department with right eye pain for several days. DIAGNOSIS: Slit lamp examination revealed the right eye cornea perforation with iris incarceration and total collapse of anterior chamber. Symblepharon formation and severe fornix shortening was also noted. While bulbi phthisis with ankyloblepharon and ocular surface keratinization was observed in the left eye. The final diagnosis was right eye stage III ocular cicatricial pemphigoid complicated with corneal perforation and iris prolapsed. INTERVENTIONS: The patient underwent ocular surface reconstruction with the aid of amniotic membrane. The first layer of the amniotic membrane was attached with tissue adhesive and fibrin glue while the second layer amniotic membrane came with a conformer ring which supported the fornix space that was recreated. Postoperative care included topical medications for inflammation alleviation. Systemic immunosuppressive agents were not administered except for oral prednisolone. OUTCOMES: No recurrence of symblepharon was noted during the one year follow-up. LESSONS: We aim at highlighting the possible important role of AMT in advance OCP. Further investigation is still needed for providing evidence to incorporate the procedure into treatment protocol.