Telangiectasia macularis eruptiva perstans (TMEP): A form of cutaneous mastocytosis with potential systemic involvement
| العنوان: | Telangiectasia macularis eruptiva perstans (TMEP): A form of cutaneous mastocytosis with potential systemic involvement |
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| المؤلفون: | Severino, Maella, Chandesris, Marie-Olivia, Barete, Stéphane, Tournier, Emilie, Sans, Béatrix, Laurent, Camille, Apoil, Pol-André, Lamant, Laurence, Mailhol, Claire, Laroche, Michel, Fraitag, Sylvie, Hanssens, Katia, Dubreuil, Patrice, Hermine, Olivier, Paul, Carle, Bulai Livideanu, Cristina |
| المساهمون: | CHU Toulouse [Toulouse], Unité différenciation épidermique et auto-immunité rhumatoïde (UDEAR), Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Centre de référence des mastocytoses (CEREMAST), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Pierre et Marie Curie - Paris 6 (UPMC), CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Institut Universitaire du Cancer de Toulouse - Oncopole (IUCT Oncopole - UMR 1037), CHU Toulouse [Toulouse]-Université Toulouse III - Paul Sabatier (UT3), Centre de Référence des Mastocytoses de Toulouse (CEREMAST), Service d'Allergologie et d'Immunologie [CHRU Toulouse], CHRU Toulouse, Service de rhumatologie et réadaptation fonctionelle, CHU Toulouse [Toulouse]-Hôpital Purpan [Toulouse], Centre de Recherche en Cancérologie de Marseille (CRCM), Aix Marseille Université (AMU)-Institut Paoli-Calmettes, Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Fédération nationale des Centres de lutte contre le Cancer (FNCLCC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université Fédérale Toulouse Midi-Pyrénées, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Université de Toulouse (UT)-Université de Toulouse (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université de Toulouse (UT)-Université de Toulouse (UT)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service d'Allergologie et d'Immunologie [CHU Toulouse], Université de Toulouse (UT) |
| المصدر: | Journal of The American Academy of Dermatology Journal of The American Academy of Dermatology, Elsevier, 2016, 74 (5), pp.885-891. ⟨10.1016/j.jaad.2015.10.050⟩ Journal of The American Academy of Dermatology, 2016, 74 (5), pp.885-891. ⟨10.1016/j.jaad.2015.10.050⟩ |
| بيانات النشر: | HAL CCSD, 2016. |
| سنة النشر: | 2016 |
| مصطلحات موضوعية: | MESH: Biopsy, Needle, MESH: Telangiectasis / pathology, telangiectasia macularis eruptiva perstans, MESH: Risk Assessment, MESH: Mastocytosis, Systemic / pathology, MESH: Prognosis, MESH: Hematologic Tests, MESH: Referral and Consultation, MESH: Sex Factors, indolent systemic mastocytosis, MESH: Severity of Illness Index, mast cell activation symptom, cutaneous mastocytosis, MESH: Cohort Studies, MESH: Age Factors, MESH: Middle Aged, mast cell disease, aggressive systemic mastocytosis, MESH: Adult, MESH: Mastocytosis, Systemic / physiopathology, MESH: Retrospective Studies, MESH: Immunohistochemistry, MESH: Telangiectasis / physiopathology, MESH: Male, MESH: Urticaria Pigmentosa / physiopathology, MESH: France, [SDV.IMM]Life Sciences [q-bio]/Immunology, MESH: Disease Progression, MESH: Urticaria Pigmentosa / epidemiology, MESH: Urticaria Pigmentosa / pathology, MESH: Mastocytosis, Systemic / epidemiology, MESH: Female |
| الوصف: | International audience; Background: Telangiectasia macularis eruptiva perstans (TMEP) has not been fully characterized.Objective: We sought to estimate the frequency and clinical characteristics of TMEP in a cohort of adult patients with cutaneous mastocytosis, and to assess the presence of systemic involvement.Methods: We included all consecutive patients evaluated for cutaneous mastocytosis in 2 centers: the Mastocytosis Competence Center of the Midi-Pyrénées from May 2006 to December 2013, and the French Reference Center for Mastocytosis from January 2008 to September 2013. Skin phenotype, histopathology, presence of KIT mutation in the skin, and assessment of systemic involvement according to World Health Organization (WHO) criteria were prospectively investigated.Results: Of 243 patients with cutaneous mastocytosis, 34 (14%) were given a diagnosis of TMEP. The diagnosis of systemic mastocytosis was established in 16 patients (47%) with TMEP. Three patients (9%) had aggressive systemic mastocytosis (C-findings according to WHO). In all, 32 patients (94%) exhibited at least 1 mast cell activation-related symptom.Limitations: Patient recruitment was undertaken at 2 referral centers with expertise in the diagnosis and treatment of mastocytosis so that the clinical findings and incidence of systemic involvement may be overestimated in comparison with the overall population of patients with TMEP.Conclusion: TMEP accounts for about 14% of patients with cutaneous mastocytosis. The disease manifests as mast cell activation symptoms in almost all patients and can be associated with systemic involvement in about 50% of cases. |
| اللغة: | English |
| تدمد: | 0190-9622 1097-6787 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=dedup_wf_001::01bbc3387fa6f605495710df8848d6b1Test https://hal-univ-tlse3.archives-ouvertes.fr/hal-03290997Test |
| رقم الانضمام: | edsair.dedup.wf.001..01bbc3387fa6f605495710df8848d6b1 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 01909622 10976787 |
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