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Journal of the American Academy of Dermatology
Volume 62, n° 3
pages 489-495 (mars 2010)
Doi : 10.1016/j.jaad.2009.04.046
Case & Review

C hronic a typical n eutrophilic d ermatosis with l ipodystrophy and e levated temperature (CANDLE) syndrome

Antonio Torrelo, MD a, , Sapna Patel, MD b, Isabel Colmenero, MD c, Dolores Gurbindo, MD d, Francisco Lendínez, MD e, Angela Hernández, MD a, Juan Carlos López-Robledillo, MD f, Ali Dadban, MD g, Luis Requena, MD h, Amy S. Paller, MD b
a Department of Dermatology, Hospital del Niño Jesús, Madrid, Spain 
b Departments of Dermatology and Pediatrics, Feinberg School of Medicine, Northwestern University, Chicago, Illinois 
c Department of Pathology, Hospital del Niño Jesús, Madrid, Spain 
d Department of Allergy and Immunology, Hospital Gregorio Marañón, Madrid, Spain 
e Department of Pediatrics, Hospital Torrecárdenas, Almería, Spain 
f Department of Rheumatology, Hospital del Niño Jesús, Madrid, Spain 
g Department of Dermatology, Amiens University Hospital, Amiens, France 
h Department of Dermatology, Fundación Jiménez Díaz, Madrid, Spain 

Reprint requests: Antonio Torrelo, MD, Department of Dermatology, Hospital del Niño Jesús, Menéndez Pelayo 65, 28009-Madrid, Spain.

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 (c hronic a typical n eutrophilic d ermatosis with l ipodystrophy and e levated temperature) syndrome for this newly described disorder, which is probably genetic in origin.

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Abbreviations used : AST, CT, ESR, NSAID

 Funding sources: None.
 Conflicts of interest: None declared.

© 2009  American Academy of Dermatology, Inc.@@#104156@@