[1]刘琼,刘金丽,廖理超,等.以荨麻疹为表现的成人Still病并发巨噬细胞活化综合征1例及文献复习[J].中国皮肤性病学杂志,2019,(05):560-563.[doi:10.13735/j.cjdv.1001-7089.201809100]
 LIU Qiong,LIU Jinli,LIAO Lichao,et al.A Case of Adult-Onset Still's Disease Complicated with Macrophage Activation Syndrome Presenting as Urticaria, and Literature Review[J].The Chinese Journal of Dermatovenereology,2019,(05):560-563.[doi:10.13735/j.cjdv.1001-7089.201809100]
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以荨麻疹为表现的成人Still病并发巨噬细胞活化综合征1例及文献复习
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《中国皮肤性病学杂志》[ISSN:1001-7089/CN:61-1197/R]

卷:
期数:
2019年05期
页码:
560-563
栏目:
病例报告
出版日期:
2019-04-01

文章信息/Info

Title:
A Case of Adult-Onset Still's Disease Complicated with Macrophage Activation Syndrome Presenting as Urticaria, and Literature Review
文章编号:
1001-7089(2019)05-0560-04
作者:
刘琼刘金丽廖理超张思平
中国科学技术大学附属第一医院(安徽省立医院)皮肤科,安徽 合肥 230001
Author(s):
LIU QiongLIU JinliLIAO LichaoZHANG Siping
(The First Affiliated Hospital of USTC,Division of Life Sciences and Medicine,University of Science and Technology of China,Hefei 230001,China)
关键词:
成人Still病巨噬细胞活化综合征皮疹
Keywords:
Adult-onset Still′s diseaseMacrophage activation syndromeRash
分类号:
R 593.2
DOI:
10.13735/j.cjdv.1001-7089.201809100
文献标志码:
B
摘要:
患者男,30岁,全身反复皮疹伴瘙痒1周,发热3 d。病程中有关节痛、咽痛,最高体温40 ℃。体检:双侧腋下可及肿大的淋巴结,颈部、躯干、四肢见红斑、风团。血常规:WBC 19.88×109/L;肝功能:ALT 525 IU/L,AST 138 IU/L;抗核抗体及类风湿因子阴性,后患者血压下降,血常规WBC 3.18×109/L,PLT 29.0×109/L;肝功能:ALT 1 520 IU/L,AST 1 107 IU/L;SF 153 042.67 ng/mL、Fib 1.06 g/L。诊断:成人Still病合并巨噬细胞活化综合征。经甲强龙、丙种球蛋白、血浆置换、环孢素等治疗,好转出院。
Abstract:
A 30-year-old man presented with one-week history of rashes and 3-day of fever, sore throat and arthralgias.Physical examination showed axillary lymphadenopathy, erythemas and wheals on the neck,trunk and limbs.Routine blood tests showed WBC19.88×109/L.liver function tests showed ALT 525 IU/L and AST138 IU/L.Both anti-nuclear antibodies and rheumatoid factors were negative.After patient′s blood pressure decreased, routine blood tests showed WBC 3.18×109/L and PLT 29.0×109/L.And liver function tests showed ALT 1 520 IU/L,AST 1 107 IU/L, SF 153 042.67 ng/mL and Fib 1.06 g/L.Patient was diagnosed with adult-onset Still′s disease and macrophage activation syndrome.Patient was discharged after treatment with methylprednisolone,intravenous immunoglobulin,plasmapheresis and cyclosporine.

参考文献/References:


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[7] Sun NZ,Brezinski EA,Berliner J,et al.Updates in adult-onset Still disease: Atypical cutaneous manifestations and associations with delayed malignancy[J].J Am Acad Dermatol,2015,73(2):294-303.
[8] Efthimiou P,Kadavath S,Mehta B.Life-threatening complications of adult-onset Still′s disease[J].Clin Rheumatol,2014,33(3):305-314.
[9] Lenert A,Yao Q.Macrophage activation syndrome complicating adult onset Still′s disease: A single center case series and comparison with literature[J].Semin Arthritis Rheum,2016,45(6):711-716.
[10] Carter SJ,Tattersall RS,Ramanan AV.Macrophage activation syndrome in adults: recent advances in pathophysiology,diagnosis and treatment[J].Rheumatology (Oxford),2018,2(22):1-13.
[11] Davi S,Consolaro A,Guseinova D,et al.An international consensus survey of diagnostic criteria for macrophage activation syndrome in systemic juvenile idiopathic arthritis[J].J Rheumatol,2011,38(4):764-768.
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备注/Memo

备注/Memo:

[作者单位]中国科学技术大学附属第一医院(安徽省立医院)皮肤科,安徽 合肥 230001
[通讯作者]张思平,E-mail:zh_siping@126.com
更新日期/Last Update: 2019-04-15