PAPA, PASH and PAPASH Syndromes: Pathophysiology, Presentation and Treatment.
Pyoderma gangrenosum (PG) is a neutrophilic dermatosis usually manifesting as skin ulcers with undermined erythematous-violaceous borders. It may be isolated, associated with systemic conditions or occurring in the context of autoinflammatory syndromes such as PAPA (pyogenic arthritis, PG and acne),...
| Publicado en: | American Journal of Clinical Dermatology Vol. 18; no. 4; pp. 555 - 563 |
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| Autores principales: | , , |
| Formato: | pictorial review tables/charts Journal Article |
| Publicado: |
Springer Nature
Aug2017
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=124090319&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 124090319 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 11750561 C4Q jtl: American Journal of Clinical Dermatology issn: 11750561 maglogo: N pubinfo: dt: Aug2017 vid: 18 iid: 4 pid: 237 pub: Springer Nature place: New York, New York artinfo: ui: 124090319 124090319 143941460 124090319 10.1007/s40257-017-0265-1 124090319 ppf: 555 ppct: 8 formats: tig: atl: PAPA, PASH and PAPASH Syndromes: Pathophysiology, Presentation and Treatment. aug: au: Cugno, Massimo Borghi, Alessandro Marzano, Angelo affil: Medicina Interna, Dipartimento di Fisiopatologia Medico-Chirurgica e dei Trapianti , Università degli Studi di Milano, Ospedale Maggiore Policlinico, Fondazione IRCCS Ca' Granda , Via Pace, 9 20122 Milan Italy sug: subj: Pyoderma Gangrenosum Diagnosis Pyoderma Gangrenosum Classification Pyoderma Gangrenosum Therapy Interleukins Immunologic Diseases Cytokines Diagnosis, Differential Treatment Outcomes Evaluation Pyoderma Gangrenosum Symptoms ab: Pyoderma gangrenosum (PG) is a neutrophilic dermatosis usually manifesting as skin ulcers with undermined erythematous-violaceous borders. It may be isolated, associated with systemic conditions or occurring in the context of autoinflammatory syndromes such as PAPA (pyogenic arthritis, PG and acne), PASH (PG, acne and suppurative hidradenitis) or PAPASH (pyogenic arthritis, acne, PG and suppurative hidradenitis). From a physiopathological point of view, all these conditions share common mechanisms consisting of over-activation of the innate immune system leading to increased production of the interleukin (IL)-1 family and 'sterile' neutrophil-rich cutaneous inflammation. From a genetic point of view, a number of mutations affecting the proteins of the inflammasome complex (the molecular platform responsible for triggering autoinflammation) or the proteins that regulate inflammasome function have been described in these disorders. As these debilitating entities are all associated with the over-expression of IL-1 and tumour necrosis factor (TNF)-α, biological drugs specifically targeting these cytokines are currently the most effective treatments but, given the emerging role of IL-17 in the pathogenesis of these syndromes, IL-17 antagonists may represent the future management of these conditions. pubtype: Academic Journal doctype: pictorial review tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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