Vulvovaginal aggressive angiomyxoma mimicking lymphedema complicated with Behcet’s disease: a case report and review of the literature

This report describes a case of aggressive angiomyxoma (AAM), arising in the vulvovaginal region, which was initially misdiagnosed as lymphedema due to a vaginal ulcer caused by Behcet’s disease. AAM is a very rare disease, usually found only in women of reproductive age, occurring predominantly in the pelviperineal region. AAM is basically a benign tumor usually treated by surgical resection. However, even after apparently complete resection, local recurrence of the tumor often occurs, thus its characterization as “aggressive” angiomyxoma. In contrast, vulvar edema is a condition with a very similar appearance that is observed relatively commonly, and which has many causes, e.g., obesity, inflammation, and benign or malignant tumors. Here we report a case of AAM which was initially mistaken for vulvovaginal edema, and initially believed to arise as a result of an ulcerative vaginal lesion associated with the patient’s underlying Behcet’s disease. We have reviewed the literature concerning vulvovaginal edema. We present herein this work to others as a precautionary tale, to prevent similar misdiagnosis and delay of treatment.