28 EyeWorld Asia Pacific | June 2024 CORNEA by Ellen Stodola, Editorial Co-Director Peripheral Ulcerative Keratitis Diagnosis and Management Peripheral Ulcerative Keratitis (PUK) is an inflammatory condition that typically affects the peripheral cornea leading to corneal thinning and corneal melt. According to Ashraf Ahmad, MD, it affects around three patients per million per year. It is commonly associated with an underlying autoimmune process, such as rheumatoid arthritis, systemic lupus, inflammatory bowel disease, or ANCA vasculitides, among others, he said. PUK is a clinical diagnosis, said Sanjay Kedhar, MD. “Patients may present with pain, redness, tearing, photophobia, or changes in vision. Slit lamp exam typically reveals a crescentic epithelial defect with stromal thinning in the peripheral cornea.” He added that in severe cases, the patient may present with a descemetocele or frank perforation of the cornea due to progressive stromal destruction and thinning. About a third of these patients will also have concurrent scleritis. “Slit lamp exams are imperative, as this is primarily a clinical diagnosis,” Dr. Ahmad said. “Exam will show a peripheral infiltrate adjacent to the limbus, often crescent shaped, with an overlying epithelial defect. Patient presentations can range from barely symptomatic to severe eye pain, redness, photophobia, and irritation.” Since PUK is typically associated with an underlying autoimmune condition, Dr. Ahmad said an appropriate review of systems should be done followed by a targeted lab workup looking for autoimmune, rheumatic, and infectious causes. Corneal culture should be considered prior to initiating antibiotics to rule out the possibility of infectious keratitis. “A thorough history and physical exam is a key component of the workup,” Dr. Kedhar said. “Cultures from the bed of the lesion should be performed if an infection is suspected. Otherwise, laboratory testing should include a complete blood count, comprehensive metabolic panel, ANCA testing, urinalysis with microscopic analysis, syphilis testing, and a chest X-ray. Additional testing such as hepatitis B triple panel test, hepatitis C antibody, TB, corneal cultures, anti-CCP, rheumatoid factor, and ANA may be ordered based on patient history and risk factors.” He added that there is a broad differential diagnosis for PUK, including corneal degenerations such as Terrien’s marginal degeneration, Fuchs superficial marginal keratitis, and pellucid marginal degeneration, as well as neoplastic etiologies such as carcinoma in situ. Left eye of a 51-year-old woman with bilateral peripheral ulcerative keratitis associated with granulomatosis with polyangiitis. Source: Sanjay Kedhar, MD Right eye of 51-year-old woman with bilateral peripheral ulcerative keratitis associated with granulomatosis with polyangiitis. Source: Sanjay Kedhar, MD
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