Periorificial cutaneous tuberculosis of the vulva.

Periorificial cutaneous tuberculosis, or tuberculosis cutis orificialis, accounts for approximately 2% of cases of tuberculosis of the skin.1 It results from the auto-inoculation of Mycobacterium tuberculosis into the periorificial oropharyngeal, anal, or genital skin or mucosa from a pulmonary, intestinal, or genitourinary focus, respectively.2,3 The definitive diagnosis is made by the isolation of M tuberculosis from the ulcer and detection of the focus of mycobacterial infection responsible for the disease. The patient was a 78-year-old woman who was referred to our outpatient clinic for mild vulvar bleeding and a longstanding painful ulcer of the labia minora of the vulva. The patient had no other symptoms. Of note in her past medical history was a cadaveric orthotopic kidney transplant in 2005 for chronic kidney failure of unknown etiology. Since that time she had been on immunosuppressive treatment with oral corticosteroids, tacrolimus, and mycophenolate mofetil. Physical examination revealed a very painful, irregular ulcer of 4× 1.5 cm on the left labium minus. The base of the ulcer was soft and erythematous and was covered with areas of adherent whitish pseudomembrane (Fig. 1). There were no palpable regional lymph nodes. Skin biopsy showed a superficial material formed of fibrin and white blood cells and deeper in the tissue there were numerous granulomas (Fig. 2), some with a necrotic center of caseous appearance, epithelioid cells, and Langhanstype multinucleated giant cells (Fig. 3). No acid-alcohol-fast bacilli were observed with Ziehl-Neelsen stain. These findings were compatible with a diagnosis of ulceration with tuberculoid granulomas. Given the presence of tuberculoid granulomas and ulceration, a request was made for screening for mycobacteria in the exudate from the ulcer and in the urine obtained by catheterization. Study of the exudate from the ulcer by polymerase chain reaction (PCR) was weakly positive. Ziehl-Neelsen stain of the sample taken from the skin was negative, but it was positive on the urine. Cultures of samples from both sites in Middlebrook 7H9 liquid medium were positive after an adequate incubation time. The microorganism isolated was identified as M tuberculosis using DNA hybridization probes. The study was completed by hysteroscopy, chest x-ray, abdominal ultrasound, and abdominal computed tomography; there were no signs of active or residual tuberculosis. The tuberculin skin test was negative. Based on these results, a diagnosis was made of vulvar tuberculosis cutis orificialis secondary to auto-inoculation from a urinary focus. The patient was immediately started on treatment with isoniazid at a dosage of 250mg/d, rifampicin, 600mg/d, pyrazinamide, 1500mg/d, and ethambutol, 800mg/d, which were continued for 2 months; she was subsequently