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dc.contributor.authorLópez-Aldabe, Kima
dc.contributor.authorEscrihuela-Vidal, Francesc
dc.contributor.authorTuells, Manel
dc.contributor.authorLlobera-Ris, Clàudia
dc.contributor.authorBauer Alonso, Andrea
dc.contributor.authorCORTES-ROMERA, MONTSERRAT
dc.contributor.authorGràcia-Sànchez, Laura
dc.contributor.authorTormo-Ratera, Marian
dc.contributor.authorJuanola Roura, Xavier
dc.contributor.authorPenin-Mosquera, Rosa Maria
dc.contributor.authorCorbella Virós, Xavier
dc.contributor.authorSolanich, Xavier
dc.date.accessioned2022-10-07T10:29:42Z
dc.date.available2022-10-07T10:29:42Z
dc.date.issued2022
dc.identifier.citationLópez-Aldabe, Kima; Escrihuela-Vidal, Francesc; Tuells, Manel [et al.]. Multiple drug regimen-refractory rosai–dorfman–destombes disease mimicking relapsing polychondritis successfully treated with cobimetinib. European Journal of Case Reports in Internal Medicine, 2022, 9(2), [p. 1-5]. Disponible en: <https://www.ejcrim.com/index.php/EJCRIM/article/view/3076>. Fecha de acceso: 7 oct. 2022. DOI: 10.12890/2022_003076ca
dc.identifier.issn2284-2594ca
dc.identifier.urihttp://hdl.handle.net/20.500.12328/3439
dc.description.abstractRosai-Dorfman-Destombes disease (RDD) or sinus histiocytosis with massive lymphadenopathy is a rare non-Langerhans cell histiocytosis of unknown cause. The disease often manifests as bilateral painless cervical lymphadenopathy associated with systemic symptoms such as fever and weight loss. Extranodal disease is also frequent and can involve any organ, mostly skin, nasal cavity, bone, and retro-orbital tissue. Swelling of cartilaginous tissues such as ear helix or laryngeal structures may mimic the entity known as relapsing polychondritis. Although spontaneous remission is the most expected evolution, some cases require systemic treatment with prednisone, methotrexate or cytotoxic agents, with variable success rates. In this respect, since somatic variants in genes involved in the mitogen-activated protein kinase (MAPK) pathway have been observed to play a pathogenic role in RDD, the use of therapies targeting these pathogenic variants seems to be a reasonable strategy. Here we present a case of RDD with extensive extranodal involvement that showed a rapid and complete response to the MEK inhibitor cobimetinib.en
dc.format.extent5ca
dc.language.isoengca
dc.publisherEuropean Federation of Internal Medicineca
dc.relation.ispartofEuropean Journal of Case Reports in Internal Medicineca
dc.relation.ispartofseries9;2
dc.relation.urihttps://www.ejcrim.com/index.php/EJCRIM/article/view/3076ca
dc.rightsEJCRIM is an open access journal, which means that all published articles are made freely available online to everyone. The costs of publication are covered by a small publication charge, which is payable by authors (or their grant funding body or institution) whose articles are accepted for publication. See also http://creativecommons.org/licenses/by-nc-nd/4.0/en
dc.rights.urihttps://creativecommons.org/licenses/by-nc-nd/4.0/
dc.subject.otherMalaltia de Rosai-Dorfman-Destombesca
dc.subject.otherMalaltia de Rosai-Dorfmanca
dc.subject.otherPolicondritis recurrentca
dc.subject.otherEnfermedad de Rosai-Dorfman-Destombeses
dc.subject.otherEnfermedad de Rosai-Dorfmanes
dc.subject.otherPolicondritis recurrentees
dc.subject.otherRosai-Dorfman-Destombes diseaseen
dc.subject.otherRosai-Dorfman diseaseen
dc.subject.otherRecurrent polychondritisen
dc.titleMultiple drug regimen-refractory rosai–dorfman–destombes disease mimicking relapsing polychondritis successfully treated with cobimetiniben
dc.typeinfo:eu-repo/semantics/articleca
dc.description.versioninfo:eu-repo/semantics/publishedVersionca
dc.rights.accessLevelinfo:eu-repo/semantics/openAccess
dc.embargo.termscapca
dc.subject.udc61ca
dc.identifier.doihttps://dx.doi.org/10.12890/2022_003076ca


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EJCRIM is an open access journal, which means that all published articles are made freely available online to everyone. The costs of publication are covered by a small publication charge, which is payable by authors (or their grant funding body or institution) whose articles are accepted for publication. See also http://creativecommons.org/licenses/by-nc-nd/4.0/
Except where otherwise noted, this item's license is described as https://creativecommons.org/licenses/by-nc-nd/4.0/
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