| Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome. | |
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MedLine Citation:
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PMID: 20159315 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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Antonio Torrelo; Sapna Patel; Isabel Colmenero; Dolores Gurbindo; Francisco Lend?nez; Angela Hern?ndez; Juan Carlos L?pez-Robledillo; Ali Dadban; Luis Requena; Amy S Paller |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: Journal of the American Academy of Dermatology Volume: 62 ISSN: 1097-6787 ISO Abbreviation: J. Am. Acad. Dermatol. Publication Date: 2010 Mar |
Date Detail:
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Created Date: 2010-02-17 Completed Date: 2010-03-23 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 7907132 Medline TA: J Am Acad Dermatol Country: United States |
Other Details:
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Languages: eng Pagination: 489-95 Citation Subset: IM |
Copyright Information:
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Copyright 2009 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved. |
Affiliation:
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Department of Dermatology, Hospital del Ni?o Jes?s, Men?ndez Pelayo 65, 28009-Madrid, Spain. atorrelo@aedv.es |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Anemia, Hypochromic
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pathology Child Child, Preschool Fatal Outcome Female Fever / pathology* Hepatomegaly / pathology Humans Infant Infant, Newborn Inflammation / pathology Lipodystrophy / pathology* Male Skin Diseases / pathology* Sweet Syndrome / diagnosis Syndrome |
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