A 26-year-old previously healthy Japanese female presented with nine days of myalgias, subjective fevers, and soaking night sweats. Four days prior, she developed multiple erythematous nodules on her face, chest, abdomen, and upper and lower extremities bilaterally. She complained of sore throat, nonproductive cough, painless oral ulcers, hand swelling, Raynaud's phenomenon, erythroderma, and abdominal bloating. She denied illicit or prescribed drug use, drug allergies, alcohol, or tobacco. She emigrated from Japan at age eight and received a BCG vaccination as a child. She reported recent travel to Europe and was sexually active with one partner. 
On physical examination, the patient was normotensive and afebrile. There was a tender right submandibular node and diffuse swelling of the hands without synovitis. Multiple 2-3 cm tender, erythematous, subcutaneous nodules were present over the face, chest, abdomen, and upper and lower extremities bilaterally (Figures 1(a) and 1(b)). She subsequently developed a fever of 39.5 Celsius. Initial laboratory tests included a hematocrit of 30.9%, an LDH of 329 iu/L, a positive Coombs antibody assay, an erythrocyte sedimentation rate of 58 mm/hr Westergren, a c-reactive protein of 182 mg/L (normal <6.3 mg/L), and a prolonged partial thromboplastin time of 38.7 sec (normal 20.9-33.6). Histochemical staining of the skin biopsy did not reveal organisms. A CT scan of the chest revealed interlobular septal and bronchial wall thickening of multiple lobes and patchy ground glass opacities suggestive of pulmonary edema or atypical or viral pneumonia. Further laboratory testing revealed an antinuclear antibody titer >640, speckled with positive anti-Smith and anti-Sm/RNP antibody assays. Complement levels and urinalysis were normal. A tuberculin skin test was positive to 14 mm at 48 hours. A quantiferon gold assay was indeterminate. A skin biopsy from her left thigh showed a relatively sparse interstitial inflammatory infiltrate composed of neutrophils, lymphocytes, and histiocytes. In addition, there were some foci of leukocytoclastic vasculitis. Neutrophils were also found in the subcutaneous tissue.
The patient was treated with prednisone 40 mg and hydroxychloroquine 400 mg daily with a dramatic response. Dapsone was later added in an effort to taper the prednisone. She was also treated for latent tuberculosis with four months of rifampin.