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Excerpt from Ulcer, CornealSynonyms, Key Words, and Related Terms: corneal melt, chronic serpiginous ulcer of the cornea, ulcus rodens, corneoscleral melting Please click here to view the full topic text: Ulcer, CornealBackground: 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. Recently, several cases of corneal melts associated with topically applied nonsteroidal anti-inflammatory drugs have been reported in the literature. Furthermore, corneoscleral melting has been reported following amniotic membrane in a patient who had undergone multiple previous ophthalmologic surgical procedures. Pathophysiology: 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. Frequency:
Mortality/Morbidity:
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