Corneal ulcer as an atypical presentation of ocular cicatricial pemphigoid.

G.B. Bietti Eye Foundation, IRCCS, Roma, Italy.
European journal of ophthalmology (Impact Factor: 1.07). 01/2007; 17(1):121-3.
Source: PubMed


To describe three patients, each presenting noninfective corneal epithelial damage as first manifestation of ocular cicatricial pemphigoid (OCP).
Case report.
Patients 1 and 2 were referred to the authors' clinic for corneal ulcer while Patient 3 for relapsed epithelial defects. All patients had negative history for systemic diseases and microbiological tests were negative. Topical steroid treatment induced the complete resolution of corneal damage. During the follow-up period, the onset of mild conjunctival fibrosis in the lower fornix allowed the authors to suspect OCP, confirmed by conjunctival biopsy.
In the three patients corneal damage was an early sign of OCP, in the absence of typical signs of conjunctival fibrosis. The authors thus suggest considering conjunctival biopsy as a useful additional test in the management of idiopathic corneal ulcers.

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    ABSTRACT: The aim of this article is to review articles on mucous membrane pemphigoid published in the English literature from 2006 to 2007. The risk of ocular disease among patients with oral only mucous membrane pemphigoid is estimated at 15-20% at 5 years. Biopsy with direct immunofluorescent testing is essential to confirm the diagnosis because histopathology even with electron microscopy does not appear to be specific enough to confirm the diagnosis of mucous membrane pemphigoid with consistency. Autoantibodies to IgG and IgG4 appear to be the most common findings observed on direct immunofluorescent testing in patients with mucous membrane pemphigoid. Treatment for mucous membrane pemphigoid typically involves immunosuppressive drugs, including biologic therapy, as well as intravenous immunoglobulin, although results have been mixed and the majority of data reported still are from case series and anecdotal evidence. Mucous membrane pemphigoid continues to be a potentially life and sight threatening disease. Therapy for this disease continues to show varied results although benefits are clearly seen in some patients. The immunosuppressive drugs used for treating mucous membrane pemphigoid and certain definitive categories of mucous membrane pemphigoid continue to evolve.
    Current opinion in ophthalmology 08/2008; 19(4):292-7. DOI:10.1097/ICU.0b013e328302cc61 · 2.50 Impact Factor


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