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Mucous Membrane Pemphigoid of the Vulva

Goldstein, Andrew T. MD*; Anhalt, Grant J. MD; Klingman, Daisy MD; Burrows, Lara J. MD§

doi: 10.1097/01.AOG.0000147837.85831.6c
Case Reports

BACKGROUND: Mucous membrane pemphigoid is a rare autoimmune blistering disease primarily affecting mucosal surfaces. Blistering and scarring may occur in the eyes, mouth, esophagus, larynx, and on the vulva. Scarring can result in severe structural changes to the vulva that may mimic the findings of other inflammatory dermatologic disorders of the vulva, including lichen sclerosus and lichen planus.

CASE: A 58-year-old woman presented with vulvar erosions, esophagitis, and laryngeal blisters. The clinical picture and the histopathology of a vulvar biopsy were suggestive of erosive lichen planus. Direct immunofluorescence, however, revealed findings diagnostic of mucous membrane pemphigoid.

CONCLUSION: This case illustrates the importance of examining extragenital mucosal surfaces of any woman presenting with vulvar lesions. In addition, it demonstrates the importance of vulvar biopsy and the usefulness of direct immunofluorescence to differentiate between conditions with similar clinical and histopathologic changes.

Direct immunofluorescence may be used to differentiate between inflammatory diseases of the vulva, such as mucous membrane pemphigoid, lichen sclerosus, and erosive lichen planus.

*Division of Gynecologic Specialties and †Department of Dermatology, The Johns Hopkins School of Medicine, Baltimore, Maryland; ‡New York University School of Medicine, New York, New York; and §Magee-Womens Hospital, the University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania

Received April 8, 2004. Received in revised form May 4, 2004. Accepted May 12, 2004.

Address reprint requests to: Andrew T. Goldstein, MD, 2002 Medical Parkway, Suite 215 Annapolis, Maryland, 21401; e-mail:

© 2005 The American College of Obstetricians and Gynecologists