Abstract
Abstract: :
Purpose: To determine to what extent patients presenting with mucous membrane pemphigoid (MMP) involving their oral mucosa, are at risk of ocular involvement. MMP is a systemic disease associated with inflammation and scarring of mucous membranes. It is unclear whether disease may remain localised to a particular anatomical site. We have noted progression of MMP to involve the ocular surface in some patients with oral MMP who were initially noted to have no ocular disease. Methods:Diagnosis of oral MMP was made on the basis of clinical signs, histology and direct and indirect immunofluorescence. Involvement of the ocular surface was graded according to the extent of conjunctival disease including vertical (II) and horizontal (III) shortening. Involvement of the ocular surface and disease progression was correlated using duration of oral disease (symptoms and signs) as an independent variable. Other variables included patient’s age, race, sex and age at diagnosis. Results:28 patients with established oral mucous membrane pemphigoid were reviewed. The mean age at diagnosis was 62.7 years (range 38yrs), female:male:46:54. At initial ocular review 7 (27%) patients showed ocular signs of pemphigoid, of whom 2 had mild [IIA IIIB], 2 moderate [IIB IIIC] and 3 severe [IIC IIID] disease respectively. Over the initial period of follow up, 2 (7%) patients developed ocular disease at 19 and 48 months, respectively, despite having had no evidence of ocular involvement at presentation. In total 39% eventually showed ocular signs over a follow–up period that ranged from 6 months to 10 years. Of the 7 patients who had initially presented with ocular disease, 22% showed progression over 1–10yrs. 61% of patients with oral MMP have not developed ocular disease. Conclusions: A significant number of our patients had ocular manifestations of their MMP, some being initially unaffected, with later involvement up to 4 years later. MMP may affect different tissues at different stages, often separated by many years. Patients with a diagnosis of oral MMP should undergo regular lifelong ophthalmic follow up, in order to detect ophthalmic manifestations at an early stage.