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Corneal Ulcer

  • Author: Fernando H Murillo-Lopez, MD; Chief Editor: Hampton Roy, Sr, MD  more...
Updated: Aug 28, 2014


This type of corneal ulcer is usually associated with a connective tissue disease, such as rheumatoid arthritis (RA), Sjögren syndrome, Mooren ulcer, or a systemic vasculitic disorder (eg, systemic lupus erythematosus [SLE], Wegener granulomatosis, polyarteritis nodosa).

RA is the most common systemic vasculitic disorder to involve the ocular surface. Patients with severe RA often present with indolent progressive ulceration of the peripheral or pericentral cornea with minimal inflammation that eventually may result in corneal perforation.

Sjögren syndrome is a particular multisystem disease that commonly occurs in middle-aged women, but it can be seen in both sexes and all ages in association with other autoimmune disorders.

Mooren ulcer is an idiopathic noninfectious ulceration of the peripheral cornea that has been classified into 2 clinical types. One is a milder, unilateral, less progressive form of the disease generally seen in elderly patients that responds well to therapy. The second type is a much more aggressive, frequently bilateral, relentless disease usually seen in younger patients that is poorly responsive to any therapy and often leads to corneal destruction.

SLE is a multisystem autoimmune disorder with ocular complications in the anterior and posterior segments, including keratitis sicca, episcleritis, corneal ulceration, uveitis, and retinal vasculitis.

Polyarteritis nodosa, Wegener granulomatosis, and scleroderma are other vasculitides that also may result in a corneal ulcer.

Polyarteritis nodosa is a vasculitis of small- and medium-sized arteries, which leads to multiple organ disease.

Wegener granulomatosis is a necrotizing, granulomatous vasculitis involving the upper respiratory tract, lungs, and kidneys. A limited form of Wegener granulomatosis exists in which renal lesions are not present.

Scleroderma is a connective tissue disorder characterized by extreme skin tautness, resulting in vascular insufficiency, vasospasm, and Raynaud phenomenon.

Paracentral corneal melting has been reported in a patient with Vogt-Koyanagi-Harada syndrome, psoriasis, and Hashimoto thyroiditis.[1]

Recently, several cases of corneal melts associated with topically applied nonsteroidal anti-inflammatory drugs have been reported in the literature.[2, 3, 4, 5] Furthermore, corneoscleral melting has been reported following amniotic membrane in a patient who had undergone multiple previous ophthalmologic surgical procedures.[6]



The pathogenesis for these corneal ulcers is not clear. Possibilities include immunologic responses to unknown antigens and genetic susceptibility, such as genetic predisposition to the development of defective suppressor T-lymphocyte function, production of autoantibodies (eg, antinuclear antibodies), and activation of the complement pathway.

Genetic and environmental factors are associated with SLE. In a genetically susceptible individual, certain environmental stimuli, such as a viral infection or contact with certain drugs, induce alterations in DNA, immunoregulatory networks, or both, with resultant formation of autoantibodies, including antinuclear antibody (ANA).

The pathogenesis of polyarteritis nodosa is not clear, but, in some patients, it may be related to hepatitis B antigen-associated immune complex disease or other immune complexes.

Recently, an association has been reported between Mooren ulcer and hepatitis C infection.[7]




United States

The incidence of corneal ulcers is uncommon.


See the list below:

  • Development of a corneal ulcer associated with a connective tissue disease or a vasculitis carries a poor prognosis.
  • Patients who have RA with scleritis and a corneal melt die within 5 years without aggressive treatment. This type of corneal ulcer may lead to corneal thinning and perforation in the perilimbal region or paracentrally.


No racial predilection exists with Wegener granulomatosis.


RA primarily affects middle-aged females.

  • Scleroderma is 3-4 times more common in women than in men.
  • Polyarteritis nodosa is 2.5 times more likely to affect males than females.
  • No sexual predilection exists with Wegener granulomatosis.


This type of corneal ulcer does not affect children. Except for the malignant form of Mooren ulcer, patients with this pathology are usually older than 30 years.

  • Wegener granulomatosis can affect all age groups.
  • Scleroderma usually starts in individuals aged 30-50 years.
  • Polyarteritis nodosa is more frequent in middle-aged males.
Contributor Information and Disclosures

Fernando H Murillo-Lopez, MD Senior Surgeon, Unidad Privada de Oftalmologia CEMES

Fernando H Murillo-Lopez, MD is a member of the following medical societies: American Academy of Ophthalmology

Disclosure: Nothing to disclose.

Specialty Editor Board

Simon K Law, MD, PharmD Clinical Professor of Health Sciences, Department of Ophthalmology, Jules Stein Eye Institute, University of California, Los Angeles, David Geffen School of Medicine

Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, Association for Research in Vision and Ophthalmology, American Glaucoma Society

Disclosure: Nothing to disclose.

J James Rowsey, MD Former Director of Corneal Services, St Luke's Cataract and Laser Institute

J James Rowsey, MD is a member of the following medical societies: American Academy of Ophthalmology, American Association for the Advancement of Science, American Medical Association, Association for Research in Vision and Ophthalmology, Florida Medical Association, Sigma Xi, Southern Medical Association, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy, Sr, MD Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy, Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

Additional Contributors

Kilbourn Gordon, III, MD, FACEP Urgent Care Physician

Kilbourn Gordon, III, MD, FACEP is a member of the following medical societies: American Academy of Ophthalmology, Wilderness Medical Society

Disclosure: Nothing to disclose.

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