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.
Department of Dermatology, Hospital del Niño Jesús, Menéndez Pelayo 65, 28009-Madrid, Spain. atorrelo@aedv.es
Antonio
A
Torrelo
Sapna
S
Patel
Isabel
I
Colmenero
Dolores
D
Gurbindo
Francisco
F
Lendínez
Angela
A
Hernández
Juan Carlos
JC
López-Robledillo
Ali
A
Dadban
Luis
L
Requena
Amy S
AS
Paller
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