Scrofuloderma: a forgotten disease?

Abstract

A 30-year-old woman was evaluated in our clinic with swelling localized to the left submandibular region and extending to the anterior part of the neck. Her symptoms first appeared 2 years earlier in the mid region of her neck. After a few months, the lesion enlarged to the left side of her neck and became crusted. She had been treated by several physicians with antibiotics, analgesics, and anti-inflammatory drugs without response. On physical examination, the patient had an axillary temperature of 38.2 degrees C. Bilateral axillary, cervical, and supraclavicular lymph nodes were detected. Dermatologic examination revealed erythematous nodules of varying sizes starting at the middle of the neck and extending to the left cervical region; an ulcerated lesion on the mid portion of the neck; and a sinus tract in the left cervical region (Figure 1). Laboratory findings included an erythrocyte sedimentation rate of 62 mm/h, but no irregularities were detected in the other hematologic and biochemical parameters. The results were negative for hepatitis markers, human immunodeficiency virus, and syphilis. A purified protein derivative test revealed an 18-mm induration with a scar of bacilli Calmette-Guérin at 48 hours. Results of chest radiography were normal, but a computerized tomographic scan of the lung revealed emphysematous changes. Acid-fast bacilli examination of the sputum was performed 3 times, all with negative results, and sputum cultures were negative for tuberculosis and deep fungal infections. Results of polymerase chain reaction testing of skin tissue, sputum, and urine were negative as well. Abdominal and pelvic ultrasonographic finding were normal; no tuberculous focus was detected from detailed examinations of pulmonary, genitourinary, and skeletal systems. Staining of a biopsy specimen obtained from lesions in the cervical region was positive for acid-fast bacilli, and cultures were positive for Mycobacterium tuberculosis. The histopathologic features of the biopsy specimen included caseation necrosis at the center and epithelioid histiocytes, lymphocytes, plasma cells, and Langhans cells at the periphery of the granulomas (Figure 2). These findings confirmed the diagnosis of scrofuloderma. The patient received multiagent (isoniazid, rifampicin, ethambutol, and pyrazinamide) antituberculosis treatment for 3 months. The treatment was continued with isoniazid, rifampicin, and ethambutol for 1 year, with follow-up clinic visits throughout the period. The lesions improved within the first 3 months of treatment (Figure 3).