Mucous membrane pemphigoid with ocular involvement
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| Year of publication | 2022 |
| Type | Conference abstract |
| MU Faculty or unit | |
| Citation | |
| Description | Introduction: Mucous membrane pemphigoid is a rare autoimmune bullous disease with an annual incidence of 1 per million, typically starting at around 60 years of age. Clinical features include erosions with subsequent scarring, especially of the mucous membranes of the oral cavity, nose, eyes and genitals. Ocular pemphigoid is a severe type of mucous membrane pemphigoid affecting the conjunctiva. Due to the rarity of the disease, low sensitivity of standard investigations (histology and immunofluorescence investigation of tissue and serum) and unavailability of serological testing of laminin 332 and alpha6beta4integrin (ELISA only for BP antigens), in many cases it is not possible to make an unequivocal diagnosis. Materials and methods: We included the patients with mucous membrane pemphigoid with ocular involvement treated at our clinic. We organized data on the current patient population, including retrospective data collection for the last 5 years with a focus on age, clinical features, investigations performed, disease course and management. The diagnosis was determined on the basis of clinical features, immunofluorescence and histological examination. Results: We enrolled 22 patients with ocular pemphigoid, of which 17 are women and 5 are men. 10 patients have only ocular involvement. 12 patients have involvement of other mucous membranes in addition to the eyes. The average age at the time of diagnosis was 68. Indirect immunofluorescence was positive in 15 patients, ELISA BP antigens were positive in 1 patient. 4 patients have a malignant disease as a comorbidity. Discussion: We created a database of patients with mucous membrane pemphigoid with the aim of creating a comprehensive overview of patients with mucous pemphigoid and to ensure their consistent management at the skin and eye clinics. Due to low sensitivity of available tests, diagnosis relies particularly on the clinical picture. Despite established therapy, we observe changes in disease activity and alternating periods of stabilization and progression. Current treatment options are able to halt disease progression, however, corticosteroid therapy and immunosuppressants can cause serious adverse effects. |