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What Is Peripheral Ulcerative Keratitis (PUK)?

Peripheral ulcerative keratitis is a destructive inflammatory condition that causes crescent shaped ulceration and thinning of the peripheral cornea near the limbus. The adjacent conjunctiva and episclera are often inflamed, and the overlying epithelium may break down. PUK is commonly associated with systemic autoimmune diseases such as rheumatoid arthritis, granulomatosis with polyangiitis, and other vasculitides. The cornea can thin to the point of impending or actual perforation, which threatens vision and globe integrity. Prompt recognition and systemic evaluation are critical.

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What Is Peripheral Ulcerative Keratitis (PUK)?

Peripheral ulcerative keratitis is a destructive inflammatory condition that causes crescent shaped ulceration and thinning of the peripheral cornea near the limbus. The adjacent conjunctiva and episclera are often inflamed, and the overlying epithelium may break down. PUK is commonly associated with systemic autoimmune diseases such as rheumatoid arthritis, granulomatosis with polyangiitis, and other vasculitides. The cornea can thin to the point of impending or actual perforation, which threatens vision and globe integrity. Prompt recognition and systemic evaluation are critical.

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Clinical Features

Patients typically present with eye pain, redness, tearing, and marked light sensitivity. On slit lamp examination, a gray white stromal infiltrate with overlying epithelial defect is seen at the peripheral cornea, often with a leading edge of active thinning. Adjacent sclera or episclera can show inflammation, and there may be associated scleritis. The central cornea is usually relatively clear early on, but progressive thinning can extend toward the visual axis. Without treatment, melting and perforation are major risks.

Systemic Associations and Pathogenesis

PUK is strongly linked to systemic collagen vascular disease, especially long standing rheumatoid arthritis, microscopic polyangiitis, and granulomatosis with polyangiitis. Immune complexes and vasculitis in the perilimbal circulation lead to ischemia and stromal destruction. Less commonly, inflammatory bowel disease, lupus, and other autoimmune disorders are implicated. Infectious keratitis can mimic PUK and sometimes coexists, so careful evaluation is needed. Laboratory studies and rheumatology consultation help uncover underlying systemic vasculitis.

Diagnosis and Differential

Diagnosis is based on the characteristic peripheral location, crescentic shape, and thinning pattern, combined with systemic history. Corneal scrapings and cultures rule out or identify superimposed infection. Imaging and blood tests, including inflammatory markers, autoantibodies, and vasculitis panels, are chosen according to suspicion. Conditions such as Mooren ulcer, infectious marginal ulcers, and simple marginal keratitis must be distinguished. Early multidisciplinary involvement improves care planning.

Treatment and Prognosis

Treatment requires aggressive systemic immunosuppression in noninfectious PUK, often starting with high dose corticosteroids followed by steroid sparing agents such as cyclophosphamide, methotrexate, or biologic drugs. Topical therapy includes lubricants and cautious use of antibiotics and steroids, with avoidance of agents that slow healing when perforation risk is high. Tissue adhesives, bandage contact lenses, or lamellar and penetrating grafts may be needed for structural support. Prognosis varies, but without early control of systemic disease, recurrence and scarring are common.

FAQs About Peripheral Ulcerative Keratitis

Is PUK just a severe form of dry eye?

No, it is a serious inflammatory and often vasculitic process that can melt the cornea, not simply surface dryness.

Does PUK always mean I have rheumatoid arthritis?

Rheumatoid arthritis is a common association, but other autoimmune and vasculitic conditions can also cause PUK.

Can PUK affect both eyes?

Yes, bilateral involvement is common in systemic disease, although one eye can be more affected than the other.

Will topical drops alone control PUK?

Topical therapy is supportive, but systemic immunosuppression is usually needed to halt tissue destruction.

References

EyeWiki. ?Peripheral Ulcerative Keratitis.? https://eyewiki.org/Peripheral_Ulcerative_Keratitis

NCBI Bookshelf (StatPearls). ?Peripheral Ulcerative Keratitis.? https://www.ncbi.nlm.nih.gov/books/NBK574556/

MSD Manual Professional Version. ?Peripheral Ulcerative Keratitis.? https://www.msdmanuals.com/professional/eye-disorders/corneal-disorders/peripheral-ulcerative-keratitis

University of Iowa EyeRounds. ?Peripheral Ulcerative Keratitis (PUK).? https://webeye.ophth.uiowa.edu/eyeforum/cases/194-PUK.htm

American Academy of Ophthalmology. ?Perforated peripheral ulcerative keratitis.? https://www.aao.org/education/image/perforated-peripheral-ulcerative-keratitis