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J Allergy Clin Immunol. 2016 Jan;137(1):35-45. doi: 10.1016/j.jaci.2015.08.034. Epub 2015 Oct 21.

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 information

1
Department of Dermatology, University of Cologne, Cologne, Germany; Department of Dermatology, University of Luebeck, Luebeck, Germany. Electronic address: karin.hartmann@uksh.de.
2
Servicio Central de Citometria (NUCLEUS), Centro de Investigacion del Cancer (IBMCC; CSIC/USAL) and Department of Medicine and IBSAL, University of Salamanca, Salamanca, Spain.
3
Norfolk and Norwich University Hospital, Norwich, United Kingdom.
4
Department of Dermatology and Allergy Biederstein, Technical University of Munich, Munich, Germany.
5
Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Md.
6
Instituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast), Hospital Virgen del Valle, Toledo, Spain.
7
Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark.
8
Department of Dermatology and Allergy, Interdisciplinary Mastocytosis Center Charité, Charité Universitätsmedizin Berlin, Berlin, Germany.
9
Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Gdansk, Poland.
10
Department of Allergology, Medical University of Gdansk, Gdansk, Poland.
11
Division of Rheumatology, Immunology, and Allergy, Harvard Medical School, Brigham and Women's Hospital, Boston, Mass.
12
Department of Allergology, Groningen Research Institute for Asthma and COPD, University Medical Center of Groningen, University of Groningen, Groningen, The Netherlands.
13
Allergy Unit, Verona University Hospital, Verona, Italy.
14
Section of Hematology, Department of Medicine, Verona University Hospital, Verona, Italy.
15
Department of Pathology, Brigham and Women's Hospital, and Harvard Medical School, Boston, Mass.
16
Department of Dermatology, Hospital del Nino Jesus, Madrid, Spain.
17
Department of Dermatology, Cantonal Hospital Aarau, Aarau, Switzerland.
18
Department of Dermatology, University of Cologne, Cologne, Germany.
19
Division of Allergic Diseases, Mayo Clinic, Rochester, Minn.
20
Division of Hematology, Department of Medicine, Stanford University School of Medicine, Stanford, Calif.
21
III. Medizinische Klinik, Universitätsmedizin Mannheim, Universität Heidelberg, Mannheim, Germany.
22
Department of Haematology, Guys and St Thomas' NHS Foundation Trust, Guys Hospital, London, United Kingdom.
23
Department of Hematology, National Reference Center of Mastocytosis, INSERM U1163, CNRS ERL8564, Imagine Institute, Université Paris Descartes, Sorbonne, Paris Cité, Paris, France.
24
Institute of Pathology, Ludwig-Maximilians-Universität, Munich, Germany.
25
Department of Pathology, University of New Mexico, Albuquerque, NM.
26
Department of Pathology, Odense University Hospital, Odense, Denmark.
27
Department of Hematology, University Hospital Groningen, University of Groningen, Groningen, The Netherlands.
28
Department of Internal Medicine, Division of Hematology, University of Istanbul, Istanbul, Turkey.
29
Hematology Center Karolinska, Karolinska University Hospital, Stockholm, Sweden.
30
Department of Internal Medicine I, Division of Hematology & Hemostaseology, Medical University of Vienna, Vienna, Austria.
31
Department of Internal Medicine, Division of Rheumatology, Allergy & Immunology, Richmond, Va.
32
Division of Allergy and Clinical Immunology, University of Salerno, Salerno, Italy.
33
Clinical Immunology and Allergy, Department of Medicine, Karolinska Institutet, Stockholm, Sweden.
34
Molecular Oncology and Pharmacology, LBPA CNRS UMR8113, Ecole Normale Supérieure de Cachan, Cachan, France.

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.

KEYWORDS:

Classification; cutaneous mastocytosis; diagnostic criteria; mast cell; mastocytosis; standardization; urticaria pigmentosa

PMID:
26476479
DOI:
10.1016/j.jaci.2015.08.034
[Indexed for MEDLINE]

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