Peripheral ulcerative keratitis: A review of aetiology and management

Roland Hӧllhumer

doi:10.4102/aveh.v81i1.697

Abstract

Background: Peripheral ulcerative keratitis (PUK) is a severe inflammatory disease of the peripheral cornea that can be caused by local factors or systemic inflammatory disease.Aim: The purpose of this review is to give an overview of the pathophysiology, aetiology, clinical features, diagnosis, and management of PUK.Method: A PubMed search was conducted using the keywords, ‘peripheral ulcerative keratitis’ and ‘Mooren’s ulcer’.Results: The peripheral cornea has unique characteristics the predispose to the development of PUK. These include fine capillary arcades that allow for deposition of immune complexes and subsequent activation of an inflammatory cascade with corneal melt. Several conditions have been implicated in the aetiology of PUK. The most commonly cited causes are rheumatoid arthritis (RA), granulomatosis with polyangiitis (GPA) and various dermatoses. In patients with RA, PUK usually presents in established disease, whereas in GPA, PUK may be the presenting feature in up to 60% of cases. In RA it heralds the onset of a systemic vasculitis with significant associated morbidity and mortality. The management of PUK follows an individualised stepwise approach. All patients require supportive measures to encourage healing and halt the process of keratolysis. Systemic autoimmune conditions need a systemic corticosteroid as a fast-acting agent to halt the inflammatory process while cytotoxic therapy maintains long term disease control. Failure to achieve disease control with CTT, necessitates the use of a biologic agent.Conclusion: Peripheral ulcerative keratitis is a severe inflammatory disease of the peripheral cornea that needs a thorough diagnostic workup and stepwise management approach.

Highlights

Peripheral ulcerative keratitis (PUK) is a rare inflammatory disease of the peripheral cornea with an incidence of 0.2–3.0 per million population per year.[1]
Peripheral ulcerative keratitis develops in the peripheral cornea because of its unique anatomical and physiological characteristics.[1]
Thickened disorganised conjunctival epithelium with mitotic squamous neoplasia figures superficial to the basal epithelium.[119] with underlying autoimmune disease, and the focus of this review is to provide an overview of the management options for this group of patients.[12,15]

Summary

Introduction

Peripheral ulcerative keratitis (PUK) is a rare inflammatory disease of the peripheral cornea with an incidence of 0.2–3.0 per million population per year.[1]. Peripheral ulcerative keratitis is an important condition as the presence of comorbid systemic disease is associated with significant morbidity and increased mortality.[2] It may be the first presentation of systemic disease, and early diagnosis and management has the potential to preserve vision and reduce mortality.[2] The purpose of this review was to provide an overview of the pathophysiology, aetiology, clinical features, diagnosis and management of PUK. A PubMed search was conducted using the keywords, ‘peripheral ulcerative keratitis’ and ‘Mooren’s ulcer’. Peripheral ulcerative keratitis (PUK) is a severe inflammatory disease of the peripheral cornea that can be caused by local factors or systemic inflammatory disease

Objectives

Results

Conclusion