Imaging findings of sterile pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome: differential diagnosis and review of the literature.
Pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome is a rare autosomal-dominant autoinflammatory disease of incomplete penetrance and variable expression. PAPA syndrome is the result of a mutation in the proline serine threonine phosphatase-interacting protein 1 (PSTPIP1/CD2BP1) gene...
| Publicado en: | Pediatric Radiology Vol. 49; no. 1; pp. 23 - 37 |
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| Autores principales: | , , , , , , |
| Formato: | diagnostic images review tables/charts Journal Article |
| Publicado: |
Springer Nature
Jan2019
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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=133859985&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 133859985 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 03010449 O03 jtl: Pediatric Radiology issn: 03010449 maglogo: N pubinfo: dt: Jan2019 vid: 49 iid: 1 pid: 237 pub: Springer Nature place: New York, New York artinfo: ui: 133859985 133859985 NLM30225645 133859985 10.1007/s00247-018-4246-1 NLM30225645 133859985 ppf: 23 ppct: 14 formats: fmt: – @attributes: type: T – @attributes: type: P tig: atl: Imaging findings of sterile pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome: differential diagnosis and review of the literature. aug: au: Martinez-Rios, Claudia Jariwala, Mehul P. Highmore, Kerri Duffy, Karen Watanabe Spiegel, Lynn Laxer, Ronald M. Stimec, Jennifer affil: Pediatric Radiology Division, Department of Medical Imaging, University of Ottawa, Children's Hospital of Eastern Ontario, 401 Smyth Road, K1H 8L1, Ottawa, ON, Canada sug: subj: Pyoderma Gangrenosum Arthritis, Infectious Acne Vulgaris ab: Pyogenic arthritis, pyoderma gangrenosum and acne (PAPA) syndrome is a rare autosomal-dominant autoinflammatory disease of incomplete penetrance and variable expression. PAPA syndrome is the result of a mutation in the proline serine threonine phosphatase-interacting protein 1 (PSTPIP1/CD2BP1) gene located on chromosome 15, which results in an abnormal overproduction of the pro-inflammatory cytokine interleukin-1β (IL-1). This syndrome clinically manifests as early onset of recurrent episodes of acute aseptic inflammation of the joints, generally occurring in the first two decades of life, followed by manifestation of characteristic skin lesions in the third decade, after an obvious decline in the joint symptoms. Although uncommon, the potential clinical implications of PAPA syndrome warrant an appropriate diagnosis in a timely fashion. pubtype: Academic Journal doctype: diagnostic images review tables/charts Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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