Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome

Antonio Torrelo*, Sapna Patel, Isabel Colmenero, Dolores Gurbindo, Francisco Lendínez, Angela Hernández, Juan Carlos López-Robledillo, Ali Dadban, Luis Requena, Amy Paller

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

122 Scopus citations

Abstract

Several syndromes manifest as recurrent daily fevers, skin lesions, and multisystem inflammation. We describe 4 patients with early-onset recurrent fevers, annular violaceous plaques, persistent violaceous eyelid swelling, low weight and height, lipodystrophy, hepatomegaly, and a range of visceral inflammatory manifestations. Laboratory abnormalities included chronic anemia, elevated acute-phase reactants, and raised liver enzymes. Histopathologic examination of lesional skin showed atypical mononuclear infiltrates of myeloid lineage and mature neutrophils. Our patients have a distinctive early-onset, chronic inflammatory condition with atypical or immature myeloid infiltrates in the skin. We propose the acronym CANDLE (chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature) syndrome for this newly described disorder, which is probably genetic in origin.

Original languageEnglish (US)
Pages (from-to)489-495
Number of pages7
JournalJournal of the American Academy of Dermatology
Volume62
Issue number3
DOIs
StatePublished - Mar 1 2010

ASJC Scopus subject areas

  • Dermatology

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