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Administration of propylthiouracil therapy has been associated with a hypersensitivity syndrome that typically manifests as vasculitis. 1 Most cases of propylthiouracil therapy-induced antineutrophil cytoplasmic antibody (ANCA) positivity react to perinuclear ANCA (p-ANCA). 1 We describe a patient who presented with cutaneous manifestations of propylthiouracil therapy hypersensitivity vasculitis with ANCA positivity.

Objective To investigate the clinical and pathologic features of patients with drug reaction with eosinophilia and systemic symptoms (DRESS) in Taiwan. Design Case series and retrospective analysis. Setting A medical referral center in Northern Taiwan. Patients Sixty cases of DRESS occurring from June 1998 to May 2008. Main outcome measures Clinical characteristics for specific drugs and important prognostic factors in DRESS. Results Patients ranged in age from 6 to 90 years (mean age, 51 years). The female to male ratio was 1.3 to 1. The most common culprit drugs were allopurinol, phenytoin, and dapsone. Exanthematous eruption was the most common skin manifestation, but purpurae and blisters were also observed. Hepatic (80%), renal (40%), and pulmonary (33%) involvement were also common. The overall mortality rate was 10%. Allopurinol-induced DRESS was characterized by preceding chronic renal insufficiency and frequent renal involvement. Pancytopenia indicated a poor prognosis. Conclusions Drug reaction with eosinophilia and systemic symptoms has a variable clinical presentation, and its definition requires clarification. It may be a heterogeneous syndrome with some particular patterns related to different drugs. Early diagnosis and prompt discontinuation of offending drug regimens are essential.

A 31-year-old Taiwanese woman, who from her medical history was generally well, presented with 1 slowly enlarging elevated plaque on the posterior aspect of her right shoulder. This had first been detected 3 months before the visit to our clinic. The lesion had originated as a small, tender, erythematous papule and had since grown into a large confluent plaque with satellite papules. She claimed that the lesion was increasing in size, and this was accompanied by intense itching and fever. On physical examination, there was 1 palm-sized, dusky red, elastic firm plaque on the right shoulder. The main lesion was surrounded by multiple 2to 8-mm erythematous satellite papules (Figure 1). There were no palpable cervical, supraclavicular, axillary, or inguinal lymph nodes. A systemic workup that included head, neck, and chest computed tomographic scans showed no involvement of other organs. Workups done to determine the source of her febrile episodes yielded no evidence of active infection. The only abnormal laboratory finding was polyclonal hypergammaglobulinemia found on serum protein electrophoresis. An incisional biopsy was performed. Microscopically, a dense dermal infiltrate extended from the upper dermis deep to the subcutis (Figure 2). The infiltrating cells were mainly composed of histiocytes with large vesicular nuclei. They also had abundant cytoplasm with spidery borders. Neutrophils, lymphocytes, and plasma cells were scattered between the histiocytes, and the presence of lymphocytes engulfed within the histiocytic cytoplasm, a feature called emperipolesis, was also noted. The mixed infiltration was neutrophil predominant. Immunohistochemical studies revealed histiocytes that stained positively for S100 protein and CD-68. Notably, the dermis surrounding the histiocytes was infiltrated by numerous neutrophils. Clinically and histologically, she was diagnosed as having cutaneous Rosai-Dorfman disease (CRD). Before visiting our hospital, she had been treated at a dermatologic clinic for 2 months, first with topical triamcinolone, oral prednisolone (30 mg/d), and then with oral isotretinoin (100 mg/d). However, the lesion had continued to worsen and the symptoms of pruritus and intermittent episodes of fever had progressed during the treatments. Superficial irradiation was suggested, but the patient refused owing to its potential hazards.