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坏疽性脓皮病发病机制与诊断的研究进展

杨岚 杨青文 付妍婕

杨岚, 杨青文, 付妍婕. 坏疽性脓皮病发病机制与诊断的研究进展[J]. 中华烧伤与创面修复杂志, 2022, 38(6): 569-573. DOI: 10.3760/cma.j.cn501225-20220331-00117.
引用本文: 杨岚, 杨青文, 付妍婕. 坏疽性脓皮病发病机制与诊断的研究进展[J]. 中华烧伤与创面修复杂志, 2022, 38(6): 569-573. DOI: 10.3760/cma.j.cn501225-20220331-00117.
Yang L,Yang QW,Fu YJ.Research advances on the pathogenesis and diagnosis of pyoderma gangrenosum[J].Chin J Burns Wounds,2022,38(6):569-573.DOI: 10.3760/cma.j.cn501225-20220331-00117.
Citation: Yang L,Yang QW,Fu YJ.Research advances on the pathogenesis and diagnosis of pyoderma gangrenosum[J].Chin J Burns Wounds,2022,38(6):569-573.DOI: 10.3760/cma.j.cn501225-20220331-00117.

坏疽性脓皮病发病机制与诊断的研究进展

doi: 10.3760/cma.j.cn501225-20220331-00117
基金项目: 

山东省自然科学基金面上项目 ZR2021MH338

详细信息
    通讯作者:

    付妍婕,Email:zcfyjzzy@163.com

Research advances on the pathogenesis and diagnosis of pyoderma gangrenosum

Funds: 

General Program of Natural Science Foundation of Shandong Province of China ZR2021MH338

More Information
    Corresponding author: Fu Yanjie, Email: zcfyjzzy@163.com
  • 摘要: 坏疽性脓皮病(PG)是一种罕见的嗜中性皮肤病,与免疫系统密切相关,其发病机制目前并不明确。PG的临床表现及组织病理学变化缺乏特异性,导致其临床诊断周期较长甚至可能被误诊,容易因此延误治疗或促使溃疡创面恶化。目前该病的诊断仍十分困难,给创面修复医师带来了极大的挑战。该文综述了近年来有关PG的病理生理、临床特征、诊断等方面的研究进展,以期为相关临床工作者提供参考。

     

  • 表1  诊断坏疽性脓皮病的PARACELSUS评分量表

    诊断标准内容标准等级标准评分(分)
    病程进展迅速主要标准3
    相关鉴别诊断的评估主要标准3
    紫红色溃疡边缘主要标准3
    对免疫抑制剂反应良好次要标准2
    典型的不规则形状溃疡次要标准2
    极度疼痛,VAS评分>4分次要标准2
    病灶位于创伤部位次要标准2
    组织病理学证实化脓性炎症附加标准1
    边缘潜行的溃疡附加标准1
    与系统性疾病相关附加标准1
    注:该表引自文献[43];PARACELSUS中各字母为各诊断标准(从上到下依次排列)英文首字母,VAS为视觉模拟评分法
    下载: 导出CSV
  • [1] Smith-PliegoM,Contreras-RuizJ,RyanS,et al.Cutaneous malakoplakia masquerading as pyoderma gangrenosum[J].Int Wound J,2017,14(4):658-660.DOI: 10.1111/iwj.12661.
    [2] LanganSM,GrovesRW,CardTR,et al.Incidence, mortality, and disease associations of pyoderma gangrenosum in the United Kingdom: a retrospective cohort study[J].J Invest Dermatol,2012,132(9):2166-2170.DOI: 10.1038/jid.2012.130.
    [3] AlaviA,FrenchLE,DavisMD,et al.Pyoderma gangrenosum: an update on pathophysiology, diagnosis and treatment[J].Am J Clin Dermatol,2017,18(3):355-372.DOI: 10.1007/s40257-017-0251-7.
    [4] Greenlee-WackerMC.Clearance of apoptotic neutrophils and resolution of inflammation[J].Immunol Rev,2016,273(1):357-370.DOI: 10.1111/imr.12453.
    [5] SatohTK,MellettM,ContassotE,et al.Are neutrophilic dermatoses autoinflammatory disorders?[J].Br J Dermatol,2018,178(3):603-613.DOI: 10.1111/bjd.15105.
    [6] Vignon-PennamenMD,WallachD.Cutaneous manifestations of neutrophilic disease. A study of seven cases[J].Dermatologica,1991,183(4):255-264.DOI: 10.1159/000247696.
    [7] MistryP,Carmona-RiveraC,OmbrelloAK,et al.Dysregulated neutrophil responses and neutrophil extracellular trap formation and degradation in PAPA syndrome[J].Ann Rheum Dis,2018,77(12):1825-1833.DOI: 10.1136/annrheumdis-2018-213746.
    [8] WangY,LiM,StadlerS,et al.Histone hypercitrullination mediates chromatin decondensation and neutrophil extracellular trap formation[J].J Cell Biol,2009,184(2):205-213.DOI: 10.1083/jcb.200806072.
    [9] SørensenOE,BorregaardN.Neutrophil extracellular traps - the dark side of neutrophils[J].J Clin Invest,2016,126(5):1612-1620.DOI: 10.1172/JCI84538.
    [10] PapayannopoulosV.Neutrophil extracellular traps in immunity and disease[J].Nat Rev Immunol,2018,18(2):134-147.DOI: 10.1038/nri.2017.105.
    [11] AdachiY,KindzelskiiAL,CookinghamG,et al.Aberrant neutrophil trafficking and metabolic oscillations in severe pyoderma gangrenosum[J].J Invest Dermatol,1998,111(2):259-268.DOI: 10.1046/j.1523-1747.1998.00311.x.
    [12] McKenzieF,ArthurM,Ortega-LoayzaAG. Pyoderma gangrenosum: what do we know now?[J]. Current Dermatology Reports,2018,7(4):147-157.DOI: 10.1007/s13671-018-0224-y.
    [13] AntonelliE,BassottiG,TramontanaM,et al.Dermatological manifestations in inflammatory bowel diseases[J].J Clin Med,2021,10(2):364.DOI: 10.3390/jcm10020364.
    [14] CugnoM,BorghiA,MarzanoAV.PAPA, PASH and PAPASH syndromes: pathophysiology, presentation and treatment[J].Am J Clin Dermatol,2017,18(4):555-562.DOI: 10.1007/s40257-017-0265-1.
    [15] PlumptreI,KnabelD,TomeckiK.Pyoderma gangrenosum: a review for the gastroenterologist[J].Inflamm Bowel Dis,2018,24(12):2510-2517.DOI: 10.1093/ibd/izy174.
    [16] HobbsMM,Ortega-LoayzaAG.Pyoderma gangrenosum: from historical perspectives to emerging investigations[J].Int Wound J,2020,17(5):1255-1265.DOI: 10.1111/iwj.13389.
    [17] MaverakisE,MarzanoAV,LeST,et al.Pyoderma gangrenosum[J].Nat Rev Dis Primers,2020,6(1):81.DOI: 10.1038/s41572-020-0213-x.
    [18] WangEA,SteelA,LuxardiG,et al.Classic ulcerative pyoderma gangrenosum is a T cell-mediated disease targeting follicular adnexal structures: a hypothesis based on molecular and clinicopathologic studies[J].Front Immunol,2018,8:1980.DOI: 10.3389/fimmu.2017.01980.
    [19] CaproniM,AntigaE,VolpiW,et al.The Treg/Th17 cell ratio is reduced in the skin lesions of patients with pyoderma gangrenosum[J].Br J Dermatol,2015,173(1):275-278.DOI: 10.1111/bjd.13670.
    [20] QuaglinoP,FavaP,CaproniM,et al.Phenotypical characterization of circulating cell subsets in pyoderma gangrenosum patients: the experience of the Italian immuno-pathology group[J].J Eur Acad Dermatol Venereol,2016,30(4):655-658.DOI: 10.1111/jdv.13100.
    [21] NesterovitchAB,GyorfyZ,HoffmanMD,et al.Alteration in the gene encoding protein tyrosine phosphatase nonreceptor type 6 (PTPN6/SHP1) may contribute to neutrophilic dermatoses[J].Am J Pathol,2011,178(4):1434-1441.DOI: 10.1016/j.ajpath.2010.12.035.
    [22] NesterovitchAB,SzantoS,GondaA,et al.Spontaneous insertion of a b2 element in the Ptpn6 gene drives a systemic autoinflammatory disease in mice resembling neutrophilic dermatosis in humans[J].Am J Pathol,2011,178(4):1701-1714.DOI: 10.1016/j.ajpath.2010.12.053.
    [23] NesterovitchAB,ArbievaZ,TothDM,et al.A differential gene expression study: Ptpn6 (SHP-1)-insufficiency leads to neutrophilic dermatosis-like disease (NDLD) in mice[J].J Dermatol Sci,2016,83(1):17-25.DOI: 10.1016/j.jdermsci.2016.03.005.
    [24] SoodAK,McShaneDB,GoogePB,et al.Successful treatment of PAPA syndrome with dual adalimumab and tacrolimus therapy[J].J Clin Immunol,2019,39(8):832-835.DOI: 10.1007/s10875-019-00685-6.
    [25] WangY,WuN,YuK,et al.Case report: pyogenic arthritis, pyoderma gangrenosum, and acne: a single-center experience and literature review[J].Front Immunol,2021,12:735851.DOI: 10.3389/fimmu.2021.735851.
    [26] MaitrepierreF,MarzanoAV,LipskerD.A unified concept of acne in the PAPA spectrum disorders[J].Dermatology,2021,237(5):827-834.DOI: 10.1159/000509874.
    [27] AhnC,NegusD,HuangW.Pyoderma gangrenosum: a review of pathogenesis and treatment[J].Expert Rev Clin Immunol,2018,14(3):225-233.DOI: 10.1080/1744666X.2018.1438269.
    [28] Alonso-LeónT,Hernández-RamírezHH,Fonte-AvalosV,et al.The great imitator with no diagnostic test: pyoderma gangrenosum[J].Int Wound J,2020,17(6):1774-1782.DOI: 10.1111/iwj.13466.
    [29] GeorgeC,DeroideF,RustinM.Pyoderma gangrenosum - a guide to diagnosis and management[J].Clin Med (Lond),2019,19(3):224-228.DOI: 10.7861/clinmedicine.19-3-224.
    [30] SullivanSN.Bullous pyoderma gangrenosum and inflammatory bowel disease[J].Inflamm Bowel Dis,2018,24(5):e16-e17.DOI: 10.1093/ibd/izy018.
    [31] ShavitE,CecchiniM,LimacherJJ,et al.Superficial granulomatous pyoderma gangrenosum involving the face: a case series of five patients and a review of the literature [formula: see text][J].J Cutan Med Surg,2021,25(4):371-376.DOI: 10.1177/1203475420988864.
    [32] AfifiL,SanchezIM,WallaceMM,et al.Diagnosis and management of peristomal pyoderma gangrenosum: a systematic review[J].J Am Acad Dermatol,2018,78(6):1195-1204.e1.DOI: 10.1016/j.jaad.2017.12.049.
    [33] PearsonWA,PrenticeDA,SinclairDL,et al.A novel topical therapy for resistant and early peristomal pyoderma gangrenosum[J].Int Wound J,2019,16(5):1136-1143.DOI: 10.1111/iwj.13164.
    [34] XuA,BalgobindA,StrunkA,et al.Prevalence estimates for pyoderma gangrenosum in the United States: an age- and sex-adjusted population analysis[J].J Am Acad Dermatol,2020,83(2):425-429.DOI: 10.1016/j.jaad.2019.08.001.
    [35] GuptaAS,Ortega-LoayzaAG.Ocular pyoderma gangrenosum: a systematic review[J].J Am Acad Dermatol,2017,76(3):512-518.DOI: 10.1016/j.jaad.2016.08.049.
    [36] ScherlingerM,GuilletS,DoutreMS,et al.Pyoderma gangrenosum with extensive pulmonary involvement[J].J Eur Acad Dermatol Venereol,2017,31(4):e214-e216.DOI: 10.1111/jdv.13976.
    [37] KridinK,CohenAD,AmberKT.Underlying systemic diseases in pyoderma gangrenosum: a systematic review and meta-analysis[J].Am J Clin Dermatol,2018,19(4):479-487.DOI: 10.1007/s40257-018-0356-7.
    [38] WallachD,Vignon-PennamenMD.Pyoderma gangrenosum and Sweet syndrome: the prototypic neutrophilic dermatoses[J].Br J Dermatol,2018,178(3):595-602.DOI: 10.1111/bjd.13955.
    [39] FloraA,KozeraE,FrewJW.Pyoderma gangrenosum: a systematic review of the molecular characteristics of disease[J].Exp Dermatol,2022,31(4):498-515.DOI: 10.1111/exd.14534.
    [40] MarzanoAV,BorghiA,WallachD,et al.A comprehensive review of neutrophilic diseases[J].Clin Rev Allergy Immunol,2018,54(1):114-130.DOI: 10.1007/s12016-017-8621-8.
    [41] LandisET,TaheriA,JorizzoJL.Gulliver's sign: a recognizable transition from inflammatory to healing stages of pyoderma gangrenosum[J].J Dermatolog Treat,2015,26(2):171-172.DOI: 10.3109/09546634.2014.883061.
    [42] SkopisM,ElghawyAA,PociurkoB,et al.Pyoderma gangrenosum associated with limited cutaneous systemic sclerosis: a rare case with literature review[J].Clin Rheumatol,2021,40(3):1141-1145.DOI: 10.1007/s10067-020-05285-z.
    [43] JockenhöferF,WollinaU,SalvaKA,et al.The PARACELSUS score: a novel diagnostic tool for pyoderma gangrenosum[J].Br J Dermatol,2019,180(3):615-620.DOI: 10.1111/bjd.16401.
    [44] MaverakisE,MaC,ShinkaiK,et al.Diagnostic criteria of ulcerative pyoderma gangrenosum: a Delphi consensus of international experts[J].JAMA Dermatol,2018,154(4):461-466.DOI: 10.1001/jamadermatol.2017.5980.
    [45] GoldustM,HagstromEL,RathodD,et al.Diagnosis and novel clinical treatment strategies for pyoderma gangrenosum[J].Expert Rev Clin Pharmacol,2020,13(2):157-161.DOI: 10.1080/17512433.2020.1709825.
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  • 收稿日期:  2022-03-31
  • 网络出版日期:  2022-08-12
  • 刊出日期:  2022-06-20

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