Blindness due to the IgA variant of epidermolysis bullosa acquisita, and treatment with osteo-odonto-keratoprosthesis

Abstract

Conflicts of interest: none declared. Sir, We describe a woman who initially presented in 1994, at age 47 years, with numerous groups of blisters in an annular arrangement on the trunk, strongly suggestive of linear IgA disease (LAD). Biopsy with immunofluorescence confirmed intense linear dermoepidermal IgA deposition which was felt to support the diagnosis of LAD, although recent re‐evaluation using indirect immunofluorescence and salt‐split skin has shown that the binding of IgA is predominantly dermal, thus suggesting that she actually has IgA‐epidermolysis bullosa acquisita (IgA‐EBA). No cause or associated disease have become apparent over 12 years of follow‐up. Some therapeutic aspects of this patient were included in an earlier report of vitamin E prophylaxis for dapsone‐induced headache1 (her initial treatment was with dapsone) but her treatments and response are further documented here as they are remarkable for the lack of efficacy. Treatments (individually or in combination) have included dapsone (with vitamin E); sulfapyridine; numerous topical (including ocular, buccal and vaginal) and systemic (oral and pulsed intravenous) corticosteroids; emollients/lubricants; topical and oral antibiotics; ciclosporin; mycophenolate mofetil; cyclophosphamide; intravenous immunoglobulin (six courses of infusions, each 2 g kg−1 over 5 days); subconjunctival mitomycin C; and subcutaneous interferon alfa (3 MIU three times weekly). Azathioprine was suggested but refused. Interferon, used on the basis of a single report in a patient who also had hepatitis C,2 was the most effective agent but caused significant anaemia and had to be discontinued.