Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome.
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.
Torrelo
Antonio
A
Department of Dermatology, Hospital del Niño Jesús, Menéndez Pelayo 65, 28009-Madrid, Spain. atorrelo@aedv.es
Patel
Sapna
S
Colmenero
Isabel
I
Gurbindo
Dolores
D
Lendínez
Francisco
F
Hernández
Angela
A
López-Robledillo
Juan Carlos
JC
Dadban
Ali
A
Requena
Luis
L
Paller
Amy S
AS
D000747:Anemia, Hypochromic / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D017809:Fatal Outcome; D005260:Female; D005334:Fever / Q000473:pathology*; D006529:Hepatomegaly / Q000473:pathology; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007249:Inflammation / Q000473:pathology; D008060:Lipodystrophy / Q000473:pathology*; D008297:Male; D012871:Skin Diseases / Q000473:pathology*; D016463:Sweet Syndrome / Q000175:diagnosis; D013577:Syndrome