Successful treatment of chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome with tofacitinib.

Pooja N Patel, Raegan Hunt, Zachary J Pettigrew, Joel Brian Shirley, Tiphanie P Vogel, Marietta M de Guzman

Pediatric dermatology 2021 Mar

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Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome is a rare autoinflammatory disorder. Cutaneous manifestations of CANDLE syndrome include characteristic recurring violaceous annular plaques comprised of an immature dermal mononuclear cell infiltrate. In CANDLE syndrome, deleterious genetic mutations inhibit proteasome-immunoproteasome function, resulting in cellular accumulation of ubiquitinated waste proteins that activate type I interferon signaling to drive inflammation. We describe a report of successful treatment of a 12-year-old girl with CANDLE syndrome with tofacitinib. © 2021 Wiley Periodicals LLC.

Citation

Pooja N Patel, Raegan Hunt, Zachary J Pettigrew, Joel Brian Shirley, Tiphanie P Vogel, Marietta M de Guzman. Successful treatment of chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome with tofacitinib. Pediatric dermatology. 2021 Mar;38(2):528-529