Mucous membrane pemphigoid of the vulva

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.

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.

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.