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 - 07/01/16
Doi : 10.1016/j.jaci.2015.08.034
Karin Hartmann, MD a, b, ⁎ 
, Luis Escribano, MD, PhD c, Clive Grattan, MA, MD d, Knut Brockow, MD e, Melody C. Carter, MD f, Ivan Alvarez-Twose, MD g, Almudena Matito, MD, PhD g, Sigurd Broesby-Olsen, MD h, Frank Siebenhaar, MD i, Magdalena Lange, MD, PhD j, Marek Niedoszytko, MD, PhD k, Mariana Castells, MD, PhD l, Joanna N.G. Oude Elberink, MD, PhD m, Patrizia Bonadonna, MD n, Roberta Zanotti, MD o, Jason L. Hornick, MD, PhD p, Antonio Torrelo, MD q, Jürgen Grabbe, MD r, Anja Rabenhorst, PhD a, Boguslaw Nedoszytko, PhD j, Joseph H. Butterfield, MD s, Jason Gotlib, MD t, Andreas Reiter, MD u, Deepti Radia, MD v, Olivier Hermine, MD, PhD w, Karl Sotlar, MD x, Tracy I. George, MD y, Thomas K. Kristensen, PhD z, Hanneke C. Kluin-Nelemans, MD, PhD aa, Selim Yavuz, MD bb, Hans Hägglund, MD, PhD cc, Wolfgang R. Sperr, MD dd, Lawrence B. Schwartz, MD, PhD ee, Massimo Triggiani, MD, PhD ff, Marcus Maurer, MD i, Gunnar Nilsson, PhD gg, Hans-Peter Horny, MD x, Michel Arock, PharmD, PhD hh, Alberto Orfao, MD, PhD c, Dean D. Metcalfe, MD f, Cem Akin, MD, PhD l, Peter Valent, MD dd
, Luis Escribano, MD, PhD c, Clive Grattan, MA, MD d, Knut Brockow, MD e, Melody C. Carter, MD f, Ivan Alvarez-Twose, MD g, Almudena Matito, MD, PhD g, Sigurd Broesby-Olsen, MD h, Frank Siebenhaar, MD i, Magdalena Lange, MD, PhD j, Marek Niedoszytko, MD, PhD k, Mariana Castells, MD, PhD l, Joanna N.G. Oude Elberink, MD, PhD m, Patrizia Bonadonna, MD n, Roberta Zanotti, MD o, Jason L. Hornick, MD, PhD p, Antonio Torrelo, MD q, Jürgen Grabbe, MD r, Anja Rabenhorst, PhD a, Boguslaw Nedoszytko, PhD j, Joseph H. Butterfield, MD s, Jason Gotlib, MD t, Andreas Reiter, MD u, Deepti Radia, MD v, Olivier Hermine, MD, PhD w, Karl Sotlar, MD x, Tracy I. George, MD y, Thomas K. Kristensen, PhD z, Hanneke C. Kluin-Nelemans, MD, PhD aa, Selim Yavuz, MD bb, Hans Hägglund, MD, PhD cc, Wolfgang R. Sperr, MD dd, Lawrence B. Schwartz, MD, PhD ee, Massimo Triggiani, MD, PhD ff, Marcus Maurer, MD i, Gunnar Nilsson, PhD gg, Hans-Peter Horny, MD x, Michel Arock, PharmD, PhD hh, Alberto Orfao, MD, PhD c, Dean D. Metcalfe, MD f, Cem Akin, MD, PhD l, Peter Valent, MD dd a Department of Dermatology, University of Cologne, Cologne, Germany
b Department of Dermatology, University of Luebeck, Luebeck, Germany
c Servicio Central de Citometria (NUCLEUS), Centro de Investigacion del Cancer (IBMCC; CSIC/USAL) and Department of Medicine and IBSAL, University of Salamanca, Salamanca, Spain
d Norfolk and Norwich University Hospital, Norwich, United Kingdom
e Department of Dermatology and Allergy Biederstein, Technical University of Munich, Munich, Germany
f Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md
g Instituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast), Hospital Virgen del Valle, Toledo, Spain
h Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark
z Department of Pathology, Odense University Hospital, Odense, Denmark
i Department of Dermatology and Allergy, Interdisciplinary Mastocytosis Center Charité, Charité Universitätsmedizin Berlin, Berlin, Germany
j Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Gdansk, Poland
k Department of Allergology, Medical University of Gdansk, Gdansk, Poland
l Division of Rheumatology, Immunology, and Allergy, Harvard Medical School, Brigham and Women's Hospital, Boston, Mass
m Department of Allergology, Groningen Research Institute for Asthma and COPD, University Medical Center of Groningen, University of Groningen, Groningen, The Netherlands
n Allergy Unit, Verona University Hospital, Verona, Italy
o Section of Hematology, Department of Medicine, Verona University Hospital, Verona, Italy
p Department of Pathology, Brigham and Women's Hospital, and Harvard Medical School, Boston, Mass
q Department of Dermatology, Hospital del Nino Jesus, Madrid, Spain
r Department of Dermatology, Cantonal Hospital Aarau, Aarau, Switzerland
s Division of Allergic Diseases, Mayo Clinic, Rochester, Minn
t Division of Hematology, Department of Medicine, Stanford University School of Medicine, Stanford, Calif
u III. Medizinische Klinik, Universitätsmedizin Mannheim, Universität Heidelberg, Mannheim, Germany
v Department of Haematology, Guys and St Thomas' NHS Foundation Trust, Guys Hospital, London, United Kingdom
w Department of Hematology, National Reference Center of Mastocytosis, INSERM U1163, CNRS ERL8564, Imagine Institute, Université Paris Descartes, Sorbonne, Paris Cité, Paris, France
x Institute of Pathology, Ludwig-Maximilians-Universität, Munich, Germany
y Department of Pathology, University of New Mexico, Albuquerque, NM
aa Department of Hematology, University Hospital Groningen, University of Groningen, Groningen, The Netherlands
bb Department of Internal Medicine, Division of Hematology, University of Istanbul, Istanbul, Turkey
cc Hematology Center Karolinska, Karolinska University Hospital, Stockholm, Sweden
dd Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria
ee Department of Internal Medicine, Division of Rheumatology, Allergy & Immunology, Richmond, Va
ff Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy
gg Clinical Immunology and Allergy, Department of Medicine, Karolinska Institutet, Stockholm, Sweden
hh Molecular Oncology and Pharmacology, LBPA CNRS UMR8113, Ecole Normale Supérieure de Cachan, Cachan, France
∗Corresponding author: Karin Hartmann, MD, Department of Dermatology, University of Luebeck, Ratzeburger Allee 160, 23538 Luebeck, Germany.
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.
Le texte complet de cet article est disponible en PDF.Key words : Classification, cutaneous mastocytosis, diagnostic criteria, mast cell, mastocytosis, standardization, urticaria pigmentosa
Abbreviations used : CM, DCM, ISM, MPCM, SM
Plan
| 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 & Immunology. A task force/consensus panel on mastocytosis between 2010 and 2012 was supported by the European Academy of Allergy and Clinical Immunology. |
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| Disclosure of potential conflict of interest: K. Hartmann has consultant arrangements with Novartis and has received payment for lectures from Abbvie, Biogen, and Novartis. F. Siebenhaar has consultant arrangements with Novartis and Patara, has received grants from Patara, and has received payment for lectures from Novartis and Uriach. M. Niedoszytko has received travel support from National Science Center Poland (2011/01/M/NZS/01362). M. Castells is on the Board of Directors for the American Academy of Allergy, Asthma & Immunology; has consultant arrangements with Merck and Sanofi; is employed by Brigham and Women's Hospital; and has received grants from Ovations for the Cure. J. N. G. Oude Elberink has consultant arrangements with HAL Allergy; has received grants from ALK-Abelló, Meda, and Chiesi; and has received payment for lectures from Novartis. A. Torrelo has consultant arrangements with Bayer and has received payment for lectures from Pierre Fabre. J. H. Butterfield has received royalties for an HMC-1 cell line. J. Gotlib has received travel support from Novartis. O. Hermine has a board membership, consultant arrangements, patents, and stock/stock options with ABScience; has received grants from Celgene; and has received travel support from Celgene, Novartis, and Roche. T. I. George has received grants from Allakos, Seattle Genetics, and Novartis; has received a consulting fee or honorarium from Novartis, Blueprint Medicine, and the Mastocytosis Society; has received travel support and fees for participation in review activities from Novartis; has consultant arrangements with Celgene and Incyte; has received payment for lectures from Sysmex; has received royalties from the American Registry of Pathology, Wolters Kluwer, and UpToDate; and has received payment for development of educational presentations from the American Society of Clinical Pathology. H. C. Kluin-Nelemans has received travel support from Novartis. W. R. Sperr has received grants from Thermo Fisher Scientific, Lipomed, Novartis, and Meda; has received travel support from Novartis, Ariad, and Gilead; has consultant arrangements with Ariad and Celgene; and has received payment for lectures from Amgen and Celgene. L. B. Schwartz has received royalties from Thermo Fisher Scientific and Millipore. A. Orfao has received grants from the Ramon Areces Foundation and Instituto de Salud Carlos III, Ministry of Economy and Competitiveness. C. Akin has consultant arrangements with Novartis, Patara Pharma, and Blueprint Medicines; has received payment for lectures from Thermo Fisher Scientific; and has a patent for the LAD2 cell line. P. Valent has consultant arrangements with Novartis; has received grants from Novartis, Celgene, Capella, and Ariad; and has received payment for lectures from Novartis, Celgene, Ariad, Pfizer, and BMS. The rest of the authors declare that they have no relevant conflicts of interest. |
© 2015
American Academy of Allergy, Asthma & Immunology. Publié par Elsevier Masson SAS. Tous droits réservés.
Vol 137 - N° 1
P. 35-45 - janvier 2016 Retour au numéro
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