Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome
Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome
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DOI：
10.1016/j.jaad.2009.04.046
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发表时间：
2010-03-01
影响因子：
13.8
通讯作者：
Paller, Amy S.
中科院分区：
文献类型：
--
作者：
Torrelo, Antonio;Patel, Sapna;Paller, Amy S.
一些综合征表现为每日反复发热、皮肤病变和多系统炎症。我们描述了4例早发性反复发热；环状紫罗兰色斑块，持续性紫罗兰色眼睑肿胀；低，体重和身高，脂肪营养不良，肝肿大，以及一系列内脏炎症表现。实验室异常包括慢性贫血、急性期反应物升高和肝酶升高。病变皮肤的组织病理学检查显示髓系和成熟中性粒细胞的非典型单核浸润。我们的患者有一个独特的早发，慢性炎症条件与非典型或未成熟的骨髓浸润在皮肤。我们提出首字母缩略词CANDLE（慢性非典型中性粒细胞性皮肤病伴脂肪营养不良和温度升高）综合征为这种新描述的疾病，这可能是遗传起源。[J] .中华皮肤科杂志，2010;32(2):489- 495。
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. (J Am Acad Dermatol 2010;62:489-95.)