dc.contributor.author | López-Aldabe, Kima | |
dc.contributor.author | Escrihuela-Vidal, Francesc | |
dc.contributor.author | Tuells, Manel | |
dc.contributor.author | Llobera-Ris, Clàudia | |
dc.contributor.author | Bauer Alonso, Andrea | |
dc.contributor.author | CORTES-ROMERA, MONTSERRAT | |
dc.contributor.author | Gràcia-Sànchez, Laura | |
dc.contributor.author | Tormo-Ratera, Marian | |
dc.contributor.author | Juanola Roura, Xavier | |
dc.contributor.author | Penin-Mosquera, Rosa Maria | |
dc.contributor.author | Corbella Virós, Xavier | |
dc.contributor.author | Solanich, Xavier | |
dc.date.accessioned | 2022-10-07T10:29:42Z | |
dc.date.available | 2022-10-07T10:29:42Z | |
dc.date.issued | 2022 | |
dc.identifier.citation | López-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_003076 | ca |
dc.identifier.issn | 2284-2594 | ca |
dc.identifier.uri | http://hdl.handle.net/20.500.12328/3439 | |
dc.description.abstract | Rosai-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.extent | 5 | ca |
dc.language.iso | eng | ca |
dc.publisher | European Federation of Internal Medicine | ca |
dc.relation.ispartof | European Journal of Case Reports in Internal Medicine | ca |
dc.relation.ispartofseries | 9;2 | |
dc.relation.uri | https://www.ejcrim.com/index.php/EJCRIM/article/view/3076 | ca |
dc.rights | 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/ | en |
dc.rights.uri | https://creativecommons.org/licenses/by-nc-nd/4.0/ | |
dc.subject.other | Malaltia de Rosai-Dorfman-Destombes | ca |
dc.subject.other | Malaltia de Rosai-Dorfman | ca |
dc.subject.other | Policondritis recurrent | ca |
dc.subject.other | Enfermedad de Rosai-Dorfman-Destombes | es |
dc.subject.other | Enfermedad de Rosai-Dorfman | es |
dc.subject.other | Policondritis recurrente | es |
dc.subject.other | Rosai-Dorfman-Destombes disease | en |
dc.subject.other | Rosai-Dorfman disease | en |
dc.subject.other | Recurrent polychondritis | en |
dc.title | Multiple drug regimen-refractory rosai–dorfman–destombes disease mimicking relapsing polychondritis successfully treated with cobimetinib | en |
dc.type | info:eu-repo/semantics/article | ca |
dc.description.version | info:eu-repo/semantics/publishedVersion | ca |
dc.rights.accessLevel | info:eu-repo/semantics/openAccess | |
dc.embargo.terms | cap | ca |
dc.subject.udc | 61 | ca |
dc.identifier.doi | https://dx.doi.org/10.12890/2022_003076 | ca |