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JAMA Clinical Challenge | Clinician's Corner

Vulvar Swelling, Plaques, and Nodules in a Young Adult Woman

Shu-li Li, MD; Chunying Li, MD, PhD
JAMA. 2013;309(24):2596-2597. doi:10.1001/jama.2013.7080.
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A 26-year-old woman presents with 5 weeks of vulvar swelling, pruritus, and progressively enlarging vulvar plaque accompanied by 2 months of occasional constipation and recurrent bloody, mucoid diarrhea. She has no history of anogenital warts and is monogamous with her husband. She has had no blood transfusions, contact with trichloroacetic acid, spermicides, or any other toxic agents or irritants. Physical examination reveals bilateral purple swelling of the labium majora and multiple moist, edematous, 1- to 8-mm wart-like skin nodules on the perineal and perianal surfaces (Figure 1). A 2-mm ulcer is observed inside the right labium minus. Digital rectal examination shows bloody mucus mixed with loose stool. White blood cell count is normal (4 × 109/L), as is neutrophil count (2.07 × 109/L); hemoglobin level is slightly decreased (10.2 g/dL). Erythrocyte sedimentation rate is 71 mm/h (reference range, 0-20 mm/h), and C-reactive protein level is 28 mg/L (reference range, 0-5 mg/L). Bacterial and fungal swab cultures from the ulceration are negative. Vaginal and urethral swabs are negative, with moderately increased polymorphonuclear leukocytes. Results of serologic testing for Treponema pallidum and human immunodeficiency virus are negative.

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Figure 1. Swelling, plaques, and nodules on the patient's vulva and perianal region
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Figure 2. Histopathology of the vulvar nodule. A, Noncaseating epithelioid granulomas in the dermis (hematoxylin-eosin, original magnification ×20). B, The granulomas were composed of multinucleated giant cells and a cellular infiltrate of lymphocytes and plasma cells (hematoxylin-eosin, original magnification ×400).
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