Knifelike Vulvar Ulcers Can Signal Crohn's Disease


HOUSTON — Women with Crohn's disease can sometimes present with knifelike vulvar ulcers that are very specific to the disorder and may be its only manifestation, according to several experts who spoke at a conference on vulvovaginal diseases jointly sponsored by Baylor College of Medicine and the Methodist Hospital.

“There are three things in the vulvar or perianal area that might make you think of unrecognized Crohn's: knifelike ulcerations, vulvar edema that has no other cause, or fistula around the anus,” said Dr. Libby Edwards, a dermatologist in private practice in Charlotte, N.C. “It has been said that vulvar Crohn's is rare, but it is not. It's uncommon but it's not rare. I see it several times a year, and most gynecologists will see it without necessarily recognizing what it is,” she said in an interview.

Although vulvar signs may be the first presentation of Crohn's for some patients, this tends to be the exception, said Dr. Hope K. Haefner, professor of obstetrics and gynecology at the University of Michigan and director of the university's Center for Vulvar Diseases in Ann Arbor.

“There is the rare patient who doesn't have any gastrointestinal symptoms and might develop them later, but usually the majority already have a diagnosis of gastrointestinal Crohn's and are seeking gynecologic care for their vulvar symptoms,” she said in an interview. In her opinion, perianal fistulae may be the most common of the three gynecologic manifestations of Crohn's. “I see them every couple of months, in children and in the elderly—there's a big age range,” she said.

Vulvar and perianal Crohn's is a marker for severe disease that needs to be aggressively treated systemically, said Dr. Edwards, also of the department of dermatology at the University of North Carolina at Chapel Hill. “First of all, if [these patients] don't have an aggressive gastroenterologist, they really need one,” she said, adding that aggressive systemic treatment for Crohn's might relieve some vulvar symptoms. But, she said, patients also need local vulvar care. “These patients get secondary infections, and when they do, they need oral antibiotics—maybe even on a long-term basis if they have open, draining sores.” In addition, both oral antibiotics and immunosuppressive therapy for Crohn's can make patients susceptible to yeast infections, which may require weekly antifungal therapy, she said.

“Knife-cut” ulcerations in skin folds are pathognomonic for vulvar Crohn's. Courtesy Dr. Libby Edwards

Fistulous tracts on the vulva are also a characteristic marker for Crohn's. Courtesy Dr. Hope K. Haefner

Perianal Crohn's consists of large anal skin tags, perianal edema, and fistulae. Courtesy Dr. Hope K. Haefner

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