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Involvement of the Female Genital Tract in Pemphigus Vulgaris

From the ¹Department of Medicine, New England Baptist Hospital, Boston, Massachusetts.

BACKGROUND: Involvement of the genital tract in women with pemphigus vulgaris has rarely been reported. We report 34 patients with pemphigus vulgaris who have involvement of the vulva, vagina, or both.

METHODS: We reviewed the clinical data on 34 women treated at a tertiary dermatology referral center from July 1988 to June 2005.

RESULTS: Of 34 patients, 21 had labial involvement, 3 had vaginal involvement, and 10 had both labial and vaginal involvement. The mean age of onset of pemphigus vulgaris was 49 years. All patients had pemphigus vulgaris involvement at multiple other sites, and all had other mucous membrane involvement, most commonly oral (97%). Recurrent episodes of genital pemphigus vulgaris were noted in 35%, with a mean of 6 recurrences (range 1–11). In all the patients the pemphigus vulgaris was controlled with systemic and local therapy. Long-term follow-up for a mean of 76 months (range 9–204) indicated that no long-term sequela occurred due to genital involvement.

CONCLUSION: Involvement of the female genital tract in pemphigus vulgaris is rare. Vulvar lesions occur more commonly than vaginal lesions. With appropriate topical and systemic therapy, patients can have full recovery with no sequelae.

LEVEL OF EVIDENCE: II-3