1. 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
- Author
-
Alberto Orfao, Cem Akin, Sigurd Broesby-Olsen, Peter Valent, Olivier Hermine, Frank Siebenhaar, Magdalena Lange, Clive Grattan, Anja Rabenhorst, Almudena Matito, Jason Gotlib, Michel Arock, Joanna N G Oude Elberink, Wolfgang R. Sperr, Melody C. Carter, Deepti Radia, Selim Yavuz, Knut Brockow, Roberta Zanotti, Karl Sotlar, Thomas Kielsgaard Kristensen, Hans Hägglund, Lawrence B. Schwartz, Bogusław Nedoszytko, Massimo Triggiani, Karin Hartmann, Hans-Peter Horny, Jason L. Hornick, Iván Álvarez-Twose, Patrizia Bonadonna, Marek Niedoszytko, Luis Escribano, Marcus Maurer, Dean D. Metcalfe, Hanneke C. Kluin-Nelemans, Antonio Torrelo, Andreas Reiter, Joseph H. Butterfield, Gunnar Nilsson, Mariana Castells, Jürgen Grabbe, Tracy I. George, Austrian Science Fund, European Academy of Allergy and Clinical Immunology, German Research Foundation, National Institute of Allergy and Infectious Diseases (US), Groningen Research Institute for Asthma and COPD (GRIAC), Stem Cell Aging Leukemia and Lymphoma (SALL), and Damage and Repair in Cancer Development and Cancer Treatment (DARE)
- Subjects
medicine.medical_specialty ,Allergy ,C-KIT MUTATIONS ,Mastocytosis, Cutaneous/classification ,Consensus ,Mastocytosis, Cutaneous ,Cutaneous mastocytosis ,Diagnostic criteria ,Solitary mastocytoma ,ONSET MASTOCYTOSIS ,DIAGNOSTIC-CRITERIA ,Diffuse cutaneous mastocytosis ,Immunology ,PEDIATRIC MASTOCYTOSIS ,Mast cell ,030207 dermatology & venereal diseases ,03 medical and health sciences ,Classification ,cutaneous mastocytosis ,diagnostic criteria ,mast cell ,mastocytosis ,standardization ,urticaria pigmentosa ,Allergy and Immunology ,Humans ,Societies, Medical ,Immunology and Allergy ,Medicine (all) ,0302 clinical medicine ,Medical ,MAST-CELL DISORDERS ,medicine ,TRYPTASE LEVELS ,ADULT PATIENTS ,Systemic mastocytosis ,GERMLINE MUTATION ,Asthma ,Urticaria pigmentosa ,Cutaneous Mastocytosis ,business.industry ,SYSTEMIC MASTOCYTOSIS ,medicine.disease ,Dermatology ,Standardization ,Telangiectasia macularis eruptiva perstans ,Cutaneous ,030228 respiratory system ,Societies ,business ,Mastocytosis - 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., Supported by research grants from the German Research Council (DFG; HA 2393/6-1; CRC/SFB832, project A14; to K.H.); from the Division of Intramural Research, National Institute of Allergy and Infectious Diseases (to D.D.M.); and from the Austrian Science Funds (FWF; projects SFB F4611 and SFB F4707-B20; to P.V.). The Consensus Conference on Mastocytosis in Boston, Massachusetts (October 2012), was supported by the American Academy of Allergy, Asthma & Immunolog. A task force/consensus panel on mastocytosis between 2010 and 2012 was supported by the European Academy of Allergy and Clinical Immunology.
- Published
- 2016
- Full Text
- View/download PDF