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Cutaneous manifestations in patients with mastocytosis: consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology

Hartmann, Karin and Escribano, Luis and Grattan, Clive and Brockow, Knut and Carter, Melody C. and Alvarez-Twose, Ivan and Matito, Almudena and Broesby-Olsen, Sigurd and Siebenhaar, Frank and Lange, Magdalena and Niedoszytko, Marek and Castells, Mariana and Oude Elberink, Joanna N. G. and Bonadonna, Patrizia and Zanotti, Roberta and Hornick, Jason L. and Torrelo, Antonio and Grabbe, Jürgen and Rabenhorst, Anja and Nedoszytko, Boguslaw and Butterfield, Joseph H. and Gotlib, Jason and Reiter, Andreas and Radia, Deepti and Hermine, Olivier and Sotlar, Karl and George, Tracy I. and Kristensen, Thomas K. and Kluin-Nelemans, Hanneke C. and Yavuz, Selim and Hägglund, Hans and Sperr, Wolfgang R. and Schwartz, Lawrence B. and Triggiani, Massimo and Maurer, Marcus and Nilsson, Gunnar and Horny, Hans-Peter and Arock, Michel and Orfao, Alberto and Metcalfe, Dean D. and Akin, Cem and Valent, Peter. (2016) Cutaneous manifestations in patients with mastocytosis: consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology. The Journal of Allergy and Clinical Immunology, 137 (1). pp. 35-45.

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Official URL: https://edoc.unibas.ch/70754/

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Abstract

Cutaneous lesions in patients with mastocytosis are highly heterogeneous and encompass localized and disseminated forms. Although a classification and criteria for cutaneous mastocytosis (CM) have been proposed, there remains a need to better define subforms of cutaneous manifestations in patients with mastocytosis. To address this unmet need, an international task force involving experts from different organizations (including the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology) met several times between 2010 and 2014 to discuss the classification and criteria for diagnosis of cutaneous manifestations in patients with mastocytosis. This article provides the major outcomes of these meetings and a proposal for a revised definition and criteria. In particular, we recommend that the typical maculopapular cutaneous lesions (urticaria pigmentosa) should be subdivided into 2 variants, namely a monomorphic variant with small maculopapular lesions, which is typically seen in adult patients, and a polymorphic variant with larger lesions of variable size and shape, which is typically seen in pediatric patients. Clinical observations suggest that the monomorphic variant, if it develops in children, often persists into adulthood, whereas the polymorphic variant may resolve around puberty. This delineation might have important prognostic implications, and its implementation in diagnostic algorithms and future mastocytosis classifications is recommended. Refinements are also suggested for the diagnostic criteria of CM, removal of telangiectasia macularis eruptiva perstans from the current classification of CM, and removal of the adjunct solitary from the term solitary mastocytoma.
Faculties and Departments:03 Faculty of Medicine > Departement Biomedizin > Department of Biomedicine, University Hospital Basel > Allergy and Immunity (Hartmann)
UniBasel Contributors:Hartmann, Karin
Item Type:Article, refereed
Article Subtype:Research Article
ISSN:0091-6749
Note:Publication type according to Uni Basel Research Database: Journal article
Identification Number:
Last Modified:17 May 2020 20:25
Deposited On:17 May 2020 20:25

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