Corneal perforation is a serious complication of peripheral ulcerative keratitis (PUK), which requires prompt control of the inflammation while restoring the integrity of the globe. This can often be challenging and may require 1 or a more surgical approaches. We report the management of 3 cases of PUK with repeated perforations and failed attempts at corneal gluing. The first case had a combination of an acute hydrops with perforation in a patient with longstanding eczema and undiagnosed keratoconus. After multiple failed attempts at gluing, a partial thickness sclerocorneal banana graft was done to restore the globe integrity. The second case had bilateral Mooren ulcer with previous perforations sealed with gluing and presented with a large peripheral perforation, which required a peripheral circular full-thickness graft fashioned with a 9.5 and a 7.5 Baron punches. The third case had psoriasis-related PUK with peripheral melting and perforation, which settled after multilayer amniotic membrane transplantation. All cases required intense control of the inflammation in conjunction with the rheumatology team. Management of PUK with perforation poses medical and surgical challenges, which can be employed as illustrated to obtain a successful outcome. Multilayer amniotic membrane transplantation can seal small perforations. Larger perforations may require fashioned banana-shaped grafts to restore the ocular integrity. Control of inflammation with systemic steroids and immunosuppression is paramount for a successful outcome.

Mooren's ulcer is a painless and idiopathic ulcer of the peripheral cornea related to autoimmunity against a corneal stromal antigen, calgranulin C. Corneal involvement is isolated. There are no specific histopathological features to differentiate Mooren's ulcer from pseudo-Mooren's, the latter being part of a systemic disease. Mooren ulcer is a diagnosis of elimination based on a complete etiological check-up. However, histopathological examination, when performed, could provide additional data to support the diagnosis. We report the case of a 78-year-old female patient who presented with Mooren's ulcer. The patient had complained of red eye and photophobia for two weeks. Initial visual acuity was "counting fingers". Clinical examination revealed a perforated perilimbic ulcer with an iris prolapse. Based on the peripheral ulcerative keratitis, with a negative etiological work-up and anatomopathological analysis of the cornea, the diagnosis of Mooren's ulcer was proposed. This rare case illustrates the need for a multidisciplinary approach involving ophthalmologists, pathologists, and internists, to reach a diagnosis and optimize the functional prognosis. Peripheral ulcerative keratitis (PUK) is a disorder affecting the juxtalimbal cornea, classically presenting with epithelial defects and stromal lysis. This rare but severe inflammatory condition results from a complex interplay between host autoimmunity, the anatomy and physiology of the peripheral cornea, and environmental factors. The underlying cause could be local or systemic, infectious or noninfectious. PUK may be due to vasculitides or collagen vascular disease, rheumatoid arthritis, granulomatosis with polyangiitis (GPA), and systemic lupus erythematosus (SLE) can account for up to 53% of PUK cases. PUK with scleritis has a poor prognosis. Progressive stromal lysis can cause corneal perforation and, in patients with an underlying autoimmune disease, carries significant morbidity and mortality. PUK without systemic association is known as Mooren ulcer (MU) and comprises 31.5% of PUK cases. Bowman first described this condition in 1849, followed by McKenzie in 1854, who called it an "ulcus roden" of the cornea. Mooren ulcer occurs in the absence of scleritis and is a diagnosis of exclusion. Clinical signs begin in the peripheral cornea and progress centrally and circumferentially, with a distinctive overhanging edge. Quick recognition of PUK is crucial, as it can be the first presenting feature of a life-threatening systemic disease. Meticulous clinical investigation and interprofessional management are required to ensure safe patient outcomes. The cornea has several layers, including the epithelium, Bowman membrane, stroma, Descemet membrane, and endothelium. The peripheral cornea has a rich vascular supply and an abundance of immune cells, making it more vulnerable to immune-mediated damage and inflammatory disorders like PUK. Most PUK cases arise in the setting of an autoimmune disorder. However, PUK may also result from infections, including herpes simplex virus and bacterial keratitis, ocular surface disorders, trauma, or surgical procedures. The natural history of PUK involves progression from peripheral corneal inflammation and thinning to ulceration, which can lead to perforation if left untreated. PUK may be staged based on the severity of corneal involvement. Without appropriate management, PUK can cause severe complications and may persist for months or even years with periods of remission and exacerbation. The pattern of spread in PUK usually involves local extension along the peripheral cornea, with the potential for centripetal spread toward the central cornea in severe cases. PUK often presents unilaterally, and bilateral involvement is associated with systemic autoimmune disease. Investigations for PUK include slit-lamp examination with fluorescein staining to evaluate epithelial defects, microbial cultures to rule out infections, and testing for systemic autoimmune markers like antinuclear antibodies (ANA), rheumatoid factor, and antineutrophil cytoplasmic antibodies (ANCA). Anterior segment optical coherence tomography may also be used to assess corneal thickness. Management of PUK involves both medical and surgical approaches. Topical steroids and immunomodulatory agents, such as cyclosporine and tacrolimus, control local inflammation, while systemic immunosuppressants, including methotrexate and biologics, are required for treating underlying autoimmune conditions. Surgical interventions, such as amniotic membrane transplantation and corneal transplantation, may be necessary in cases of severe corneal damage or perforation. Referral to a rheumatologist for managing associated systemic diseases is essential to ensure long-term control and prevent recurrence. The prognosis for PUK varies with severity and the timeliness of treatment. If detected early and treated aggressively, patients can have a favorable outcome and preserve vision. However, delayed treatment may result in corneal perforation, permanent vision loss, and secondary infections.

To report an unusual case of bilateral aggressive Mooren ulcer that occurred in the setting of bilateral pterygia and showed a relentless course during pregnancy. A 39-year-old woman of Black African ethnicity, 36-week pregnant, presented to the eye casualty with bilateral nasal corneal ulcer and associated melt around preexisting pterygia. A detailed workup including microbial evaluation, culture and sensitivity, polymerase chain reaction for herpes simplex virus, varicella zoster virus, and cytomegalovirus, inflammatory blood profile, autoimmune markers, and human leucocyte antigen (HLA) screening was undertaken. Treatment was initiated in a stepwise approach. Infections and systemic autoimmune and rheumatologic conditions were ruled out. A diagnosis of bilateral Mooren ulcer was made by exclusion. The peripheral blood was positive for HLA DQ2. As the condition seemed refractory to medical management (topical steroids and intravenous pulse methylprednisolone followed by oral prednisolone and topical cyclosporine), urgent bilateral conjunctival resection with multilayered amniotic membrane transplantation was performed to reduce the inflammatory stimulus and keratolysis. Stabilization of the condition warranted the need for systemic immunosuppressive agents. Using a multidisciplinary approach, in liaison with Obstetricians and Rheumatologists, the patient was planned for an earlier elective Cesarean section and commencement of oral mycophenolate mofetil postpartum, which aided in successful control of the disease. Mooren ulcer could follow an aggressive course during pregnancy, especially in the setting of preexisting pterygium. The complex hormonal and immunological changes during pregnancy and the delivery of inflammatory mediators directly onto the cornea by pterygium could contribute to the severity. A well-planned, stepwise, and multidisciplinary management is pivotal for the treatment of this condition.