PUK from diagnosis to treatment

Purpose To describe the treatment strategy in the management of peripheral ulcerative keratitis (PUK). Methods The current literature is reviewed and the experience of a tertiary referral centre is reported. Results Peripheral ulcerative keratitis (PUK) is rare but severe sight-threatening disease affecting the peripheral cornea. The causes of PUK are multiple and insidious. Infectious diseases, both secondary to systemic rather than purely local diseases, and non-infectious diseases can be identified as a cause of PUK. Non-infectious systemic diseases, which can induce deposition of immune complexes in the cornea and, hence, lead to corneal ulcers, include peripheral vasculitides and inflammatory diseases of collagen, such as Rheumathoid arthritis, Wegener Granulomatosis and Systemic Lupus Erythematosus. Mooren’s ulcer is one of the most characteristic PUKs: often unilateral and self-limiting in the elderly, sometimes bilateral and relentless in young patients, may cause severe visual impairment following extensive corneal destruction. Immunosuppressive therapy has been demonstrated effective in improving the prognosis of progressive cases, although a certain number of patients remain refractory to treatment. Resolution of refractory cases of Mooren’s ulcer with new systemic biologic agents, such as campath-1H and anti-tumor necrosis factor (TNF)-α, has been reported. Conclusion PUK can be a severe disease, leading to significant visual impairment. Although no guideline is provided, the current medical literature can give the basis for a successful treatment strategy. The detection of the infectious trigger can lead to the correct, specific therapy. Non-infectious diseases are basically treated with the combination of steroids with immunesuppresives and, when necessary, biologics.