TY - JOUR
T1 - Cutaneous manifestations in patients with mastocytosis
T2 - 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
AU - Hartmann, Karin
AU - Escribano, Luis
AU - Grattan, Clive
AU - Brockow, Knut
AU - Carter, Melody C.
AU - Alvarez-Twose, Ivan
AU - Matito, Almudena
AU - Broesby-Olsen, Sigurd
AU - Siebenhaar, Frank
AU - Lange, Magdalena
AU - Niedoszytko, Marek
AU - Castells, Mariana
AU - Oude Elberink, Joanna N.G.
AU - Bonadonna, Patrizia
AU - Zanotti, Roberta
AU - Hornick, Jason L.
AU - Torrelo, Antonio
AU - Grabbe, Jürgen
AU - Rabenhorst, Anja
AU - Nedoszytko, Boguslaw
AU - Butterfield, Joseph H.
AU - Gotlib, Jason
AU - Reiter, Andreas
AU - Radia, Deepti
AU - Hermine, Olivier
AU - Sotlar, Karl
AU - George, Tracy I.
AU - Kristensen, Thomas K.
AU - Kluin-Nelemans, Hanneke C.
AU - Yavuz, Selim
AU - Hägglund, Hans
AU - Sperr, Wolfgang R.
AU - Schwartz, Lawrence B.
AU - Triggiani, Massimo
AU - Maurer, Marcus
AU - Nilsson, Gunnar
AU - Horny, Hans Peter
AU - Arock, Michel
AU - Orfao, Alberto
AU - Metcalfe, Dean D.
AU - Akin, Cem
AU - Valent, Peter
N1 - Publisher Copyright:
© 2015 American Academy of Allergy, Asthma & Immunology.
PY - 2016/1/1
Y1 - 2016/1/1
N2 - 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.
AB - 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.
KW - Classification
KW - cutaneous mastocytosis
KW - diagnostic criteria
KW - mast cell
KW - mastocytosis
KW - standardization
KW - urticaria pigmentosa
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U2 - 10.1016/j.jaci.2015.08.034
DO - 10.1016/j.jaci.2015.08.034
M3 - Article
C2 - 26476479
AN - SCOPUS:84953637134
SN - 0091-6749
VL - 137
SP - 35
EP - 45
JO - Journal of Allergy and Clinical Immunology
JF - Journal of Allergy and Clinical Immunology
IS - 1
ER -