- Author: Fernando H Murillo-Lopez, MD; Chief Editor: Hampton Roy, Sr, MD more...
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 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.
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.
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.
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.
Paroli MP, Pinca M, Speranza S, Marino M, Pivetti-Pezzi P. Paracentral corneal melting in a patient with Vogt-Koyanagi-Harada's syndrome, psoriasis, and Hashimoto's thyroiditis. Ocul Immunol Inflamm. 2003 Dec. 11(4):309-13. [Medline].
Asai T, Nakagami T, Mochizuki M. Three cases of corneal melting after instillation of a new nonsteroidal anti-inflammatory drug. Cornea. 2006 Feb. 25 (2):224-7. [Medline].
Flach AJ. Corneal melts associated with topically applied nonsteroidal anti-inflammatory drugs. Trans Am Ophthalmol Soc. 2001. 99:205-10; discussion 210-2. [Medline].
Guidera AC, Luchs JI, Udell IJ. Keratitis, ulceration, and perforation associated with topical nonsteroidal anti-inflammatory drugs. Ophthalmology. 2001 May. 108(5):936-44. [Medline].
Lambiase A, Bonini S, Aloe L, et al. Anti-inflammatory and healing properties of nerve growth factor in immune corneal ulcers with stromal melting. Arch Ophthalmol. 2000 Oct. 118(10):1446-9. [Medline].
Schechter BA, Rand WJ, Nagler RS. Corneal melt after amniotic membrane transplant. Cornea. 2005 Jan. 24(1):106-7. [Medline].
Wilson SE, Lee WM, Murakami C, Weng J, Moninger GA. Mooren-type hepatitis C virus-associated corneal ulceration. Ophthalmology. 1994 Apr. 101(4):736-45. [Medline].
Yazici AT, Kara N, Yüksel K, Altinkaynak H, Baz O, Bozkurt E, et al. The biomechanical properties of the cornea in patients with systemic lupus erythematosus. Eye (Lond). 2011 Aug. 25(8):1005-9. [Medline]. [Full Text].
Vanathi M, Sharma N, Titiyal JS. Tectonic grafts for corneal thinning and perforations. Cornea. 2002 Nov. 21(8):792-7. [Medline].
Foster CS, Forstot SL, Wilson LA. Mortality rate in rheumatoid arthritis patients developing necrotizing scleritis or peripheral ulcerative keratitis. Effects of systemic immunosuppression. Ophthalmology. 1984 Oct. 91(10):1253-63. [Medline].
Bullen CL, Liesegang TJ, McDonald TJ, DeRemee RA. Ocular complications of Wegener's granulomatosis. Ophthalmology. 1983 Mar. 90(3):279-90. [Medline].
Lambiase A, Rama P, Bonini S, et al. Topical treatment with nerve growth factor for corneal neurotrophic ulcers. N Engl J Med. 1998 Apr 23. 338(17):1174-80. [Medline].
Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997 Sep. 40(9):1725. [Medline].
Jayson MI, Jones DE. Scleritis and rheumatoid arthritis. Ann Rheum Dis. 1971 Jul. 30(4):343-7. [Medline].
Robin JB, Schanzlin DJ, Meisler DM, et al. Ocular involvement in the respiratory vasculitides. Surv Ophthalmol. 1985 Sep-Oct. 30(2):127-40. [Medline].
West RH, Barnett AJ. Ocular involvement in scleroderma. Br J Ophthalmol. 1979 Dec. 63(12):845-7. [Medline].