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Clinical Characteristics of Mucous Membrane Pemphigoid and Pseudopemphigoid

¹ Ophthalmology, The Johns Hopkins University School of Medicine, Baltimore, MD, United States
² Dermatology, The Johns Hopkins University School of Medicine, Baltimore, MD, United States
³ Ophthalmology and Medicine, The Johns Hopkins University School of Medicine, Baltimore, MD, United States

Commercial Relationships: J.E. Thorne, None; G.J. Anhalt, None; D.A. Jabs, None.

Grant Identification: Support: NIH Grants EY13707 (Dr. Thorne), AI48063 (Dr. Anhalt), EY00405

Abstract

Purpose: To describe the demographic and clinical characteristics of patients with mucous membrane pemphigoid (MMP) and pseudopemphigoid. To quantitate the risk of developing ocular MMP among patients who presented with MMP without ocular involvement.
Methods: A retrospective cohort study was performed. Information on 280 consecutive patients that presented for evaluation of possible MMP from January 1, 1985 to December 31, 2001 was entered prospectively into a database. The database was supplemented by a retrospective chart review. MMP was diagnosed in patients with cicatrizing conjunctivitis who had either: 1) linear deposition of antibodies to the basement membrane zone (BMZ) on direct immunofluorescent analysis of a mucous membrane biopsy specimen; or 2) the presence of circulating autoantibodies to epithelial BMZ.
Results: Extraocular disease was common in patients who presented with ocular MMP, and primarily involved the oropharynx (84.8% of patients presenting with ocular MMP). Nasal (51.9%), cutaneous (34.2%), esophageal (20.3%), and laryngeal (6.3%) disease also were seen at the time of presentation. The risk of ocular involvement for patients with extraocular MMP presenting without ocular disease was 5% per year. The cumulative risk of developing ocular involvement at five years of follow-up was 22%. Using direct immunofluorescent techniques, the initial conjunctival biopsy was positive for MMP in 77% of patients diagnosed with ocular MMP. The three most frequent causes of pseudopemphigoid were topical glaucoma medications (28.3%), rosacea blepharoconjunctivitis (20.0%), and atopic keratoconjunctivitis (8.3%).
Conclusions: Patients who present with ocular MMP typically have other systemic manifestations of MMP. Those patients who present with extraocular MMP without ocular involvement are at risk for developing ocular disease. Ocular MMP and pseudopemphigoid are similar clinically; therefore immunohistologic evaluation of biopsied tissue is necessary to confirm the diagnosis of MMP before treatment is instituted.

Keywords: autoimmune disease • conjunctivitis • inflammation

© 2003, The Association for Research in Vision and Ophthalmology, Inc., all rights reserved. For permission to reproduce any part of this abstract, contact the ARVO Office at arvo{at}arvo.org.