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      Histopathological characteristics are instrumental to distinguish monomorphic from polymorphic maculopapular cutaneous mastocytosis in children

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          Abstract

          Background

          Mastocytosis is characterized by the accumulation of mast cells (MCs) in the skin or other organs, and can manifest at any age. A significant number of paediatric mastocytosis cases persist after puberty. In particular, monomorphic maculopapular cutaneous mastocytosis (mMPCM) is often persistent and associated with systemic mastocytosis. However, clinical differentiation of MPCM from polymorphic (p)MPCM can be difficult.

          Aim

          To identify histopathological features that can help to distinguish mMPCM from other subtypes of paediatric mastocytosis.

          Methods

          This was a retrospective study using skin biopsies from patients with any subtype of mastocytosis. The localization and density of the MC infiltrate, MC morphology and expression of aberrant markers were evaluated and correlated with clinical characteristics.

          Results

          In total, 33 biopsies were available for evaluation from 26 children [(10 with mMPCM, 5 with mastocytoma, 3 with diffuse cutaneous mastocytosis (DCM), 8 with pMPCM)] and 7 adults with MPCM. The MC number was increased in all patients, but was higher in children than adults ( P < 0.01). The presence of mMPCM was associated with sparing of the papillary dermis from MC infiltration, whereas MC density in the papillary dermis was highest in pMPCM and DCM ( P < 0.01). The positive predictive value of the presence of a reticular MC infiltrate for mMPCM was 72.7% (95% CI 51.4–87.0), and the negative predictive value was 83.3% (95% CI 42.2–97.2). There were no relevant differences in the expression of CD2, CD25 or CD30 between the different subtypes.

          Conclusion

          Skin histopathology might enhance the phenotypical differentiation of mMPCM from other subtypes in children, thereby increasing the accuracy of one's prognosis.

          Abstract

          In this study, we found that paediatric monomorphic maculopapular cutaneous mastocytosis (mMPCM) was associated with a specific histopathological pattern compared with other paediatric subtypes of mastocytosis such as polymorphic (p)MPCM. Paediatric mMPCM was characterized by a predominantly reticular situated MC infiltrate with sparing of the papillary dermis. This was similar to the pattern seen in adult‐onset MPCM; however, total MC counts in lesional skin were significantly higher in children compared with adults. Histopathology might be a useful addition to the clinical phenotype and laboratory parameters to distinguish mMPCM from pMPCM in children.

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          Most cited references24

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          Mastocytosis 2016: Updated WHO Classification and Novel Emerging Treatment Concepts.

          Over the past few years substantial advances have been made in understanding the pathogenesis, evolution, and complexity of mast cell neoplasms. New diagnostic and prognostic parameters and novel therapeutic targets with demonstrable clinical impact have been identified. A number of these new markers, molecular targets, and therapeutic approaches have been validated and translated into clinical practice. At the same time, the classification of mastocytosis and related diagnostic criteria have been refined and updated by the consensus group and the World Health Organization (WHO). As a result, more specific therapies tailored towards prognostic sub-groups of patients have been developed. Emerging treatment concepts employ drugs directed against KIT and other relevant targets in neoplastic mast cells, and will hopefully receive recognition by health authorities in the near future. The current article provides an overview of recent developments in the field, with emphasis on the updated WHO classification, refined criteria, additional prognostic parameters, and novel therapeutic approaches. Based on these emerging concepts, the prognosis, quality of life, and survival of patients with advanced mastocytosis are expected to improve in the coming years.
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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.

            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.
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              Paediatric mastocytosis: a systematic review of 1747 cases.

              Paediatric mastocytosis was previously considered to be a benign and spontaneously regressing disease. However, this evolution is impossible to predict. To clarify the characteristics and course of paediatric mastocytosis, we performed a literature review of 1747 cases published between 1950 and April 2014. Lesions occurred before the age of 2 years in 90% of cases, and presented as urticaria pigmentosa (75% of cases), mastocytoma (20%) or diffuse cutaneous mastocytosis (5%). The male-to-female ratio was 1·4. KIT D816V mutation was detected in 34% of 215 tested patients. Clinical regression (complete or partial) occurred in 67% of cases and stabilization in 27%. However, the outcome was fatal in 2·9% of patients.
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                Author and article information

                Contributors
                m.hermans@erasmusmc.nl
                Journal
                Clin Exp Dermatol
                Clin Exp Dermatol
                10.1111/(ISSN)1365-2230
                CED
                Clinical and Experimental Dermatology
                John Wiley and Sons Inc. (Hoboken )
                0307-6938
                1365-2230
                11 July 2022
                September 2022
                : 47
                : 9 ( doiID: 10.1111/ced.v47.9 )
                : 1694-1702
                Affiliations
                [ 1 ] Section of Allergy and Clinical Immunology Department of Internal Medicine Erasmus University Medical Center Rotterdam The Netherlands
                [ 2 ] Department of Dermatology Erasmus University MC Sophia Children's Hospital Rotterdam The Netherlands
                [ 3 ] Section of Allergy Department of Paediatric Medicine Erasmus University MC Sophia Children's Hospital Rotterdam The Netherlands
                [ 4 ] Department of Pathology Erasmus University MC Rotterdam The Netherlands
                [ 5 ] Department of Paediatric Haematology Erasmus University MC Sophia Children's Hospital Rotterdam The Netherlands
                [ 6 ] Department of Immunology Erasmus University MC Rotterdam The Netherlands
                Author notes
                [*] [* ] Correspondence: Dr Maud A. W. Hermans, Deparment of Allergy and Clinical Immunology, Erasmus Medical Center, Dr Molewaterplein 40, Rotterdam 3015, GD, The Netherlands

                E‐mail: m.hermans@ 123456erasmusmc.nl

                Author information
                https://orcid.org/0000-0002-1643-8387
                Article
                CED15262 CED-2021-1754.R2
                10.1111/ced.15262
                9544455
                35596520
                c3c9c0a9-0b19-4530-a7ca-59b11c63b9a4
                © 2022 The Authors. Clinical and Experimental Dermatology published by John Wiley & Sons Ltd on behalf of British Association of Dermatologists.

                This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

                History
                : 17 May 2022
                Page count
                Figures: 5, Tables: 2, Pages: 1702, Words: 5468
                Categories
                Original Article
                Original Articles
                Custom metadata
                2.0
                September 2022
                Converter:WILEY_ML3GV2_TO_JATSPMC version:6.2.0 mode:remove_FC converted:07.10.2022

                Dermatology
                Dermatology

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