MOOREN ULCER

pathogenesis although the etiology of puk is unknown, evidence is mounting that autoimmunity plays a key role. the following have been found in patients with mooren ulcer: deficiency of suppressor t cells increased level of iga increased concentration of plasma cells and lymphocytes in the conjunctiva adjacent to the ulcerated areas increased cd4+/cd8+ and b7-2+/antigen-presenting cell ratios as well as increased vcam - i, vla-4, and icam -i in the vascular endothelium of conjunctival vessels tissue-fixed immunoglobulins and complement in the conjunctival epithelium and peripheral cornea A significant number of resident cells in Mooren ulcer specimens express MHC class II antigens, a reflection of the degree of immune-mediated i nflarnmation in the tissue. It has been suggested that auto reactivity to a cornea-specific antigen may playa role in the pathogenesis of th is disorder, and humoral and cell -mediated immune mechanisms may be involved in the init iation and perpetuation of corneal des truction. The proximity of the ulcerative lesion to the limbus probably has pathophysiologic im portance (as discussed earlier in the sectio n on PUK), because resection or recession of the limbal conjunctiva can often have a beneficial therapeutic effect. Although the cause of Mooren ulcer is unknown, precipitati ng factors include accidental trauma or surgery and exposure to parasitic infection. The latter is of considerable importance, as th e incidence of Mooren ulcer is particularly high in areas where parasitic (eg. helmi nthic) infections are endemic. The principal hypotheses are that inflammation associated with previous injury or infection may alter the expression of corneal or conjunctival antigens (to which autoantibodies are then produced) or that cross-reactivity occurs between the i.m rnune effectors generated in response to infection and corneal autoantigens. The simultaneous presence of multiple types of inflammatory cell s. adhesion. and co stimulatory molecules in Mooren ulcer conjunctiva suggests that their interaction may contribute to a sustained immune activation as at least part of the pathogenic mechanism of this disorder. By definition, Mooren ulcer is of

unknown cause. Cases of PUK due to known local (eg. rosacea) or systemic (eg. rheumatoid arthrit is) diseases should not be called Mooren ulcer.

CLINICAL PRESENTATION Mom-en ulcer is a chronic, progressive, painful, idiopathic ulceration of the peripheral corneal stroma and epithelium. Typicall y. the ulcer starts in the periphery of the cornea and spreads circumfe rentially and then centripetall y. with a leading underm ined edge of deepithelized tissue (Fig 7-19). A slower movement of ulceration proceeds toward the sclera. The eye is inflamed and pain can be intense. with photophobia and tea ri ng. Perforation may occur with minor t rauma or during secondary infection. Extensive vascularization and fibrosis of the cornea may occur. In some patients. it may be very diffi cult to di stinguish Mooren ulcer from idiopathic PUK. An important distinguishi ng feature is the purely corneal involvement of Momen; PUK has scleral involvement. Two clinical types of Momen ulcer have been described. Unilateral Mooren ulcer typically occu rs in an older patient population. Sex distribution is equal in this form, which is slowly progressive. A second type of Mooren ulcer is more common in Africa. This form is usually bilateral, rapidly progressive, and poorly responsive to medical or surgical intervention (Fig 7-20). Corneal ulceration and perfo ration are frequ ent. Many of the patients with this form of Mooren ulcer also have coexist ing parasitemia. It is possible that in this subgroup of West African males, Mooren ulcer may be triggered by antigen- antibody reaction to helminthic toxins or antigens deposited in the limbal cornea during the bloodborne phase of parasitic infection.

MANAGEMENT The multitude of therapeutic strategies used against Mooren ulcer un derscores the relative lack of effec tive treatment. Topical corticosteroids, contact lenses, acetyleysteine (Mucomyst 10%) and L-cysteine (0.2 molar), topical cyciosporine, limbal conjunctival excision, and lamellar ke ratoplasty have all been described with variable success. More recently, topical IFN-a" and topical cyclosporine 2%, as well as infliximab, have also been reported as effective alternat ives. Systemic immunosuppressives such as oral corticosteroids, cyclophosphamide, methotrexate, and cyclosporine have also shown promise in these cases. Hepatit is C-associated cases of "Mooren ulcer" -type PUK have responded to in terferon therapy.