关键词: CANDLE syndrome PRAAS baricitinib

来  源:   DOI:10.1111/pde.15667

Abstract:
Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) or proteasome-associated autoinflammatory syndrome is a rare autoinflammatory disorder that typically presents in infancy with characteristic symptoms, including recurrent fever, panniculitis, and progressive lipodystrophy, among other findings. We present a case of mother and child with CANDLE syndrome. The child was eventually started on baricitinib with normalization of rash and systemic findings.
摘要:
慢性非典型中性粒细胞性皮肤病伴脂肪营养不良和体温升高(CANDLE)或蛋白酶体相关的自身炎症综合征是一种罕见的自身炎症性疾病,通常在婴儿期出现特征性症状,包括反复发烧,脂膜炎,和进行性脂肪营养不良,在其他发现中。我们介绍了一个母亲和孩子患有CANLE综合征的病例。该孩子最终开始使用baricitinib,皮疹和全身性发现正常化。
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