Mucous membrane pemphigoid and pseudopemphigoid.
OBJECTIVE
To describe the clinical characteristics of patients with mucous membrane pemphigoid (MMP) and pseudopemphigoid.


METHODS
Retrospective cohort study.


METHODS
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.


METHODS
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.
Thorne
Jennifer E
JE
Department of Ophthalmology, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21205, USA.
Anhalt
Grant J
GJ
Jabs
Douglas A
DA
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood; D001324:Autoantigens / Q000276:immunology; D001485:Basement Membrane / Q000276:immunology; D001706:Biopsy; D002352:Carrier Proteins; D002648:Child; D002675:Child, Preschool; D003094:Collagen / Q000276:immunology; D003176:Complement C3 / Q000276:immunology; D003228:Conjunctiva / Q000276:immunology / Q000473:pathology*; D003231:Conjunctivitis / Q000175:diagnosis* / Q000276:immunology; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D008297:Male; D008875:Middle Aged; D009092:Mucous Membrane / Q000473:pathology; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D010390:Pemphigoid, Benign Mucous Membrane / Q000175:diagnosis* / Q000276:immunology; D012189:Retrospective Studies; D000097805:Collagen Type XVII
D001323:Autoantibodies; D001324:Autoantigens; D002352:Carrier Proteins; D003094:Collagen; D003176:Complement C3; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII; C486268:DST protein, human