CANDLE Syndrome

What is the chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome?

Patients with CANDLE syndrome have recurrent fever in infancy, almost daily, in addition to skin rash (erythematous annular plaques) and unique facial features including lipodystrophy (loss of fat on their face). It is associated with a genetic mutation in the PSMB8 gene that encodes components of cellular proteasomes resulting in an abnormal interferon-γ response. See Table 76.1 for additional details regarding this condition.

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