Parts of this article were accepted for presentation at: American Academy of Ophthalmology Annual Meeting, November, 2003; Anaheim, California.
Received 25 September 2002; accepted 21 March 2003.
Abstract
Purpose
To describe the clinical characteristics of patients with mucous membrane pemphigoid (MMP) and pseudopemphigoid.
Design
Retrospective cohort study.
Participants
Two hundred eighty consecutive patients referred for the evaluation of possible ocular MMP from January 1, 1985, to December 31, 2001.
Methods
Information on patients presenting for evaluation of possible MMP was entered prospectively into a database, which was supplemented by a retrospective chart review. Mucous membrane pemphigoid was diagnosed in patients with a compatible clinical picture by the linear deposition of antibodies to the basement membrane zone (BMZ) on direct immunofluorescent analysis of a mucous membrane biopsy specimen or by the presence of circulating autoantibodies to epithelial BMZ.
Main outcome measures
Demographic and clinical characteristics of MMP and pseudopemphigoid; risk of ocular MMP among patients presenting with extraocular MMP without ocular disease.
Results
Among patients with ocular MMP, extraocular disease was common (82.4% of patients). The risk of ocular involvement among patients with MMP seen without ocular disease was approximately 5% per year over the first 5 years of follow-up (cumulative risk at 5 years, 22%). Although immunohistologic confirmation of the diagnosis was obtained in all patients, the initial conjunctival biopsy was positive for MMP in 80% of the patients diagnosed with ocular MMP. The most frequent presumed causes of pseudopemphigoid were topical glaucoma medications (28.3%), rosacea blepharoconjunctivitis (20.0%), atopic keratoconjunctivitis (8.3%), and conjunctival lichen planus (8.3%).
Conclusions
Patients with ocular MMP typically have other systemic manifestations of MMP. Patients who are initially seen with extraocular MMP without ocular involvement are at risk for ocular disease developing. The clinical characteristics of ocular MMP and pseudopemphigoid are similar; therefore, immunohistologic evaluation of biopsied tissue is needed to confirm the diagnosis of MMP.
1Department of Ophthalmology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
2Department of Dermatology, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
3Department of Medicine, The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
4Department of Epidemiology, The Johns Hopkins University Bloomberg School of Public Health, Baltimore, Maryland, USA
Correspondence and reprint requests to Douglas A. Jabs, MD, MBA, The Wilmer Eye Institute, 550 North Broadway, Suite 700, Baltimore, MD 21205, USA.
Supported in part by grants K23 EY13707 (JET), R01 AI 48063 (GJA), and K24 EY00405 (DAJ) from the National Institutes of Health, Bethesda, Maryland. Dr Jabs is the recipient of a Research to Prevent Blindness Senior Investigator Award.
ABSTRACT