Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome.
J Am Acad Dermatol
; 62(3): 489-95, 2010 Mar.
Article
em En
| MEDLINE
| ID: mdl-20159315
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.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Dermatopatias
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Febre
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Lipodistrofia
Tipo de estudo:
Diagnostic_studies
Limite:
Child
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Child, preschool
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Female
/
Humans
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Infant
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Male
/
Newborn
Idioma:
En
Revista:
J Am Acad Dermatol
Ano de publicação:
2010
Tipo de documento:
Article
País de afiliação:
Espanha