EyeWorld is the official news magazine of the American Society of Cataract & Refractive Surgery.
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84 | EYEWORLD | SPRING 2024 C ORNEA by Ellen Stodola Editorial Co-Director About the physicians Ashraf Ahmad, MD Cataract, Cornea & Refractive Surgery Harvard Eye Associates Laguna Hills, California Sanjay Kedhar, MD Clinical Professor of Ophthalmology Gavin Herbert Eye Institute University of California, Irvine Irvine, California P eripheral ulcerative keratitis (PUK) is an inflammatory condition that typical- ly affects the peripheral cornea leading to corneal thinning and corneal melt. According to Ashraf Ahmad, MD, it af- fects around three patients per million per year. It is commonly associated with an underlying autoimmune process, such as rheumatoid arthri- tis, 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 cres- centic epithelial defect with stromal thinning in the peripheral cornea." He added that in severe cases, the patient may present with a descem- etocele or frank perforation of the cornea due to progressive stromal destruction and thinning. About a third of these patients will also have a concurrent scleritis. "Slit lamp exam is 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 look- ing 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. Ked- har 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 microscop- ic 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 degenera- tions such as Terrien's marginal degeneration, Fuchs superficial marginal keratitis, and pellu- cid marginal degeneration, as well as neoplastic etiologies such as carcinoma in situ. Dr. Ahmad said there may be some chal- lenges with confirming a diagnosis because many other conditions can mimic PUK. He ex- panded on the differential diagnosis to also in- clude infectious keratitis, herpes viral keratitis, and staph marginal keratitis. "Differentiating between these entities can be challenging and requires a careful history and physical exam," he said. To treat PUK, Dr. Ahmad said patients typically require systemic steroids to control Peripheral ulcerative keratitis diagnosis and management Left eye of 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