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Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature: The CANDLE syndrome


Four case reports of chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature-CANDLE syndrome, previously unreported, were described. Three females (aged 2, 10 and 14 years) and one 8-year-old male presented with symptoms including recurrent fevers, annular violaceous plaques, chronic anemia, lipodystrophy and neutrophilic dermatosis. Treatment (mainly unsuccessful) comprised nonsteroidal anti-inflammatory drugs, colchicine, prednisone, methotrexate, etanercept, ciclosporin, rituximab, azathioprine, immunoglobulin and dapsone. The 14-year-old female died. CANDLE disorder is thought to be genetic in origin, but further studies are needed to elucidate its etiology and treatment (Torrelo, A. et al. J Am Acad Dermatol 2010, 62(3): 489).

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