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成人Still病合并中毒性表皮坏死松解症1例并文献回顾

Toxic Epidermal Necrolysis in A Patient with Adult-onset Still Disease and Literature Review
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摘要 患者女,31岁。全身无瘙痒性红斑伴发热3周。实验室检查:铁蛋白>1 500ng/m L。入院后排除感染、肿瘤和结缔组织病,诊断成人Still病。患者入院后经糖皮质激素联合高剂量静脉丙种球蛋白治疗后好转。在病情稳定10d后予激素减量。减量后第2天面胸部、肩背部新发瘙痒性红斑,继而出现水疱及皮肤大片松解。追问病史患者在入院前2周曾使用"吲哚美辛栓",诊断为成人Still病合并中毒性表皮坏死松解症。在激素不加量情况下再次联合使用静脉丙种球蛋白,患者皮损得以控制,出院后随访2年激素已停药,皮损无复发。 A 31-year-old female presented with widespread asymptomatic erythema with spiking fever for 3 weeks. Laboratory tests showed elevated serum ferritin (higher than 1 500ng/mL). Excluding infections, malignancies and connective tissue diseases, the diagnosis of adult-onset Still disease (AOSD) was made. The symptoms were controlled with systemic corticosteroid and high dose intravenous immunoglobulin (IVIg). Ten days after her symptoms resolved,the dose of corticosteroid reduced. Two days after reducing dose of corticosteroid, the patient had pruritic erythema on her trunk,which quickly evolving to bullae and epidermolysis. The patient recalled that she had taken indometacin two weeks before admission. The diagno- sis of toxic epidermal necrolysis (TEN) was made. IVIg was administered again and her lesions resolved. After 2 years follow-up, she stopped corticosteroid and still had no lesion.
出处 《中国皮肤性病学杂志》 CAS CSCD 北大核心 2015年第9期943-945,共3页 The Chinese Journal of Dermatovenereology
关键词 成人STILL病 中毒性表皮坏死松解症 静脉丙种球蛋白 Aduh onset Still's disease TEN IVIg
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参考文献14

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二级参考文献55

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