Pyoderma gangrenosum in a patient with familial Mediterranean fever and chronic inflammatory seronegative arthropathy: a unique triad.
Familial Mediterranean fever (FMF) is an autoinflammatory disorder characterised by recurrent fever attacks and serositis. Chronic inflammatory seronegative arthropathy affects the spine and peripheral joints and rarely coexists with FMF. Pyoderma gangrenosum (PG) is a neutrophilic dermatosis that m...
| Publicado en: | BMJ Case Reports Vol. 15; no. 7; pp. 1 - 4 |
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| Autores principales: | , |
| Formato: | Journal Article |
| Publicado: |
BMJ Publishing Group
Jul2022
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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=158375625&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 158375625 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 1757790X B755 jtl: BMJ Case Reports issn: 1757790X maglogo: N pubinfo: dt: Jul2022 vid: 15 iid: 7 pid: 8280 pub: BMJ Publishing Group artinfo: ui: 158375625 10.1136/bcr-2022-250060 158375625 ppf: 1 ppct: 3 formats: tig: atl: Pyoderma gangrenosum in a patient with familial Mediterranean fever and chronic inflammatory seronegative arthropathy: a unique triad. aug: au: Constantinou, Maria Parperis, Konstantinos affil: Medical School, University of Cyprus Medical School, Nicosia, Cyprus. sug: ab: Familial Mediterranean fever (FMF) is an autoinflammatory disorder characterised by recurrent fever attacks and serositis. Chronic inflammatory seronegative arthropathy affects the spine and peripheral joints and rarely coexists with FMF. Pyoderma gangrenosum (PG) is a neutrophilic dermatosis that manifests as an ulcerative skin disease that uncommonly occurs in patients with FMF. In this case report, we describe a male patient in his 60s with a history of FMF and chronic inflammatory seronegative arthropathy who developed ulcerative skin lesions consistent with PG. A genetic evaluation revealed a pathogenic variant (V726A) and two variants of uncertain significance (F479L and E167D) mutations in the MEFV gene. We hypothesised that the triad of FMF, chronic inflammatory seronegative arthropathy and PG might be linked to the V726A variant, while the presence of the other two variants may have amplified the clinical presentation. Further studies are warranted to confirm our observation. pubtype: Academic Journal doctype: Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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