Histopathological characteristics are instrumental to distinguish monomorphic from polymorphic maculopapular cutaneous mastocytosis in children
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 per...
Gespeichert in:
| Veröffentlicht in: | Clinical and experimental dermatology 2022-09, Vol.47 (9), p.1694-1702 |
|---|---|
| Hauptverfasser: | , , , , , , , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | 1702 |
|---|---|
| container_issue | 9 |
| container_start_page | 1694 |
| container_title | Clinical and experimental dermatology |
| container_volume | 47 |
| creator | Hermans, Maud A. W. Pasmans, Suzanne G. M. A. Arends, Nicolette J. T. Bosch, Thierry P. P. Daele, Paul L. A. Doorn, Martijn B. A. Huisman, Elise J. Mooyaart, Antien L. Damman, Jeffrey |
| description | 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 |
| doi_str_mv | 10.1111/ced.15262 |
| format | Article |
| fullrecord | <record><control><sourceid>proquest_cross</sourceid><recordid>TN_cdi_proquest_miscellaneous_2667788128</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><sourcerecordid>2703902028</sourcerecordid><originalsourceid>FETCH-LOGICAL-c3532-433a8e2103502b9d5afb0767aa7f8e3dde0977627ad222b865bd072039576f23</originalsourceid><addsrcrecordid>eNp1kc1q3DAURkVpSKZJFn2BYuimWXgiXVWSvSzTpBMIZJO9kGV5RkG2XP1Q5inyylXGmU2h2gi-ezj3wofQZ4LXpLxbbfo1YcDhA1oRylkNQPFHtMIUi5q3tLlAn2J8wZhQItg5uqCMtZwBXqHXrY3JzyrtvfM7q5Wr9F4FpZMJZWJ1rFQwlZ1iCnk0UypA8lX_Npt22cZ9NfrJjz7Me6urIfixmr07nIJR6eyKf85OhUrnpCbjcyx5WasPyUcbi70sta4PZrpCZ4Ny0Vy__5fo-f7uebOtH59-PWx-PNaaMgr1d0pVY4BgyjB0bc_U0GHBhVJiaAzte4NbITgI1QNA13DW9VgApi0TfAB6ib4t2jn439nEJEcbtXFuOU8C50I0DYGmoF__QV98DlM5ToIoQgz4SN0slA4-xmAGOQc7qnCQBMu3kmQpSR5LKuyXd2PuxpKeyFMrBbhdgD_WmcP_TXJz93NR_gX8eJ75</addsrcrecordid><sourcetype>Aggregation Database</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>2703902028</pqid></control><display><type>article</type><title>Histopathological characteristics are instrumental to distinguish monomorphic from polymorphic maculopapular cutaneous mastocytosis in children</title><source>Oxford University Press Journals All Titles (1996-Current)</source><source>Alma/SFX Local Collection</source><creator>Hermans, Maud A. W. ; Pasmans, Suzanne G. M. A. ; Arends, Nicolette J. T. ; Bosch, Thierry P. P. ; Daele, Paul L. A. ; Doorn, Martijn B. A. ; Huisman, Elise J. ; Mooyaart, Antien L. ; Damman, Jeffrey</creator><creatorcontrib>Hermans, Maud A. W. ; Pasmans, Suzanne G. M. A. ; Arends, Nicolette J. T. ; Bosch, Thierry P. P. ; Daele, Paul L. A. ; Doorn, Martijn B. A. ; Huisman, Elise J. ; Mooyaart, Antien L. ; Damman, Jeffrey</creatorcontrib><description>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.
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.</description><identifier>ISSN: 0307-6938</identifier><identifier>EISSN: 1365-2230</identifier><identifier>DOI: 10.1111/ced.15262</identifier><identifier>PMID: 35596520</identifier><language>eng</language><publisher>England: Oxford University Press</publisher><subject>Biopsy ; CD25 antigen ; CD30 antigen ; Children ; Dermis ; Localization ; Mast cells ; Mastocytoma ; Mastocytosis ; Patients ; Pediatrics ; Puberty ; Skin ; Skin cancer</subject><ispartof>Clinical and experimental dermatology, 2022-09, Vol.47 (9), p.1694-1702</ispartof><rights>2022 The Authors. published by John Wiley & Sons Ltd on behalf of British Association of Dermatologists.</rights><rights>This article is protected by copyright. All rights reserved.</rights><rights>2022. This article is published under http://creativecommons.org/licenses/by/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c3532-433a8e2103502b9d5afb0767aa7f8e3dde0977627ad222b865bd072039576f23</citedby><cites>FETCH-LOGICAL-c3532-433a8e2103502b9d5afb0767aa7f8e3dde0977627ad222b865bd072039576f23</cites><orcidid>0000-0002-1643-8387</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27901,27902</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/35596520$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Hermans, Maud A. W.</creatorcontrib><creatorcontrib>Pasmans, Suzanne G. M. A.</creatorcontrib><creatorcontrib>Arends, Nicolette J. T.</creatorcontrib><creatorcontrib>Bosch, Thierry P. P.</creatorcontrib><creatorcontrib>Daele, Paul L. A.</creatorcontrib><creatorcontrib>Doorn, Martijn B. A.</creatorcontrib><creatorcontrib>Huisman, Elise J.</creatorcontrib><creatorcontrib>Mooyaart, Antien L.</creatorcontrib><creatorcontrib>Damman, Jeffrey</creatorcontrib><title>Histopathological characteristics are instrumental to distinguish monomorphic from polymorphic maculopapular cutaneous mastocytosis in children</title><title>Clinical and experimental dermatology</title><addtitle>Clin Exp Dermatol</addtitle><description>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.
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.</description><subject>Biopsy</subject><subject>CD25 antigen</subject><subject>CD30 antigen</subject><subject>Children</subject><subject>Dermis</subject><subject>Localization</subject><subject>Mast cells</subject><subject>Mastocytoma</subject><subject>Mastocytosis</subject><subject>Patients</subject><subject>Pediatrics</subject><subject>Puberty</subject><subject>Skin</subject><subject>Skin cancer</subject><issn>0307-6938</issn><issn>1365-2230</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2022</creationdate><recordtype>article</recordtype><sourceid>24P</sourceid><recordid>eNp1kc1q3DAURkVpSKZJFn2BYuimWXgiXVWSvSzTpBMIZJO9kGV5RkG2XP1Q5inyylXGmU2h2gi-ezj3wofQZ4LXpLxbbfo1YcDhA1oRylkNQPFHtMIUi5q3tLlAn2J8wZhQItg5uqCMtZwBXqHXrY3JzyrtvfM7q5Wr9F4FpZMJZWJ1rFQwlZ1iCnk0UypA8lX_Npt22cZ9NfrJjz7Me6urIfixmr07nIJR6eyKf85OhUrnpCbjcyx5WasPyUcbi70sta4PZrpCZ4Ny0Vy__5fo-f7uebOtH59-PWx-PNaaMgr1d0pVY4BgyjB0bc_U0GHBhVJiaAzte4NbITgI1QNA13DW9VgApi0TfAB6ib4t2jn439nEJEcbtXFuOU8C50I0DYGmoF__QV98DlM5ToIoQgz4SN0slA4-xmAGOQc7qnCQBMu3kmQpSR5LKuyXd2PuxpKeyFMrBbhdgD_WmcP_TXJz93NR_gX8eJ75</recordid><startdate>202209</startdate><enddate>202209</enddate><creator>Hermans, Maud A. W.</creator><creator>Pasmans, Suzanne G. M. A.</creator><creator>Arends, Nicolette J. T.</creator><creator>Bosch, Thierry P. P.</creator><creator>Daele, Paul L. A.</creator><creator>Doorn, Martijn B. A.</creator><creator>Huisman, Elise J.</creator><creator>Mooyaart, Antien L.</creator><creator>Damman, Jeffrey</creator><general>Oxford University Press</general><scope>24P</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>K9.</scope><scope>7X8</scope><orcidid>https://orcid.org/0000-0002-1643-8387</orcidid></search><sort><creationdate>202209</creationdate><title>Histopathological characteristics are instrumental to distinguish monomorphic from polymorphic maculopapular cutaneous mastocytosis in children</title><author>Hermans, Maud A. W. ; Pasmans, Suzanne G. M. A. ; Arends, Nicolette J. T. ; Bosch, Thierry P. P. ; Daele, Paul L. A. ; Doorn, Martijn B. A. ; Huisman, Elise J. ; Mooyaart, Antien L. ; Damman, Jeffrey</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c3532-433a8e2103502b9d5afb0767aa7f8e3dde0977627ad222b865bd072039576f23</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2022</creationdate><topic>Biopsy</topic><topic>CD25 antigen</topic><topic>CD30 antigen</topic><topic>Children</topic><topic>Dermis</topic><topic>Localization</topic><topic>Mast cells</topic><topic>Mastocytoma</topic><topic>Mastocytosis</topic><topic>Patients</topic><topic>Pediatrics</topic><topic>Puberty</topic><topic>Skin</topic><topic>Skin cancer</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Hermans, Maud A. W.</creatorcontrib><creatorcontrib>Pasmans, Suzanne G. M. A.</creatorcontrib><creatorcontrib>Arends, Nicolette J. T.</creatorcontrib><creatorcontrib>Bosch, Thierry P. P.</creatorcontrib><creatorcontrib>Daele, Paul L. A.</creatorcontrib><creatorcontrib>Doorn, Martijn B. A.</creatorcontrib><creatorcontrib>Huisman, Elise J.</creatorcontrib><creatorcontrib>Mooyaart, Antien L.</creatorcontrib><creatorcontrib>Damman, Jeffrey</creatorcontrib><collection>Wiley Online Library Open Access</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>MEDLINE - Academic</collection><jtitle>Clinical and experimental dermatology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Hermans, Maud A. W.</au><au>Pasmans, Suzanne G. M. A.</au><au>Arends, Nicolette J. T.</au><au>Bosch, Thierry P. P.</au><au>Daele, Paul L. A.</au><au>Doorn, Martijn B. A.</au><au>Huisman, Elise J.</au><au>Mooyaart, Antien L.</au><au>Damman, Jeffrey</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Histopathological characteristics are instrumental to distinguish monomorphic from polymorphic maculopapular cutaneous mastocytosis in children</atitle><jtitle>Clinical and experimental dermatology</jtitle><addtitle>Clin Exp Dermatol</addtitle><date>2022-09</date><risdate>2022</risdate><volume>47</volume><issue>9</issue><spage>1694</spage><epage>1702</epage><pages>1694-1702</pages><issn>0307-6938</issn><eissn>1365-2230</eissn><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.
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.</abstract><cop>England</cop><pub>Oxford University Press</pub><pmid>35596520</pmid><doi>10.1111/ced.15262</doi><tpages>1702</tpages><orcidid>https://orcid.org/0000-0002-1643-8387</orcidid><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0307-6938 |
| ispartof | Clinical and experimental dermatology, 2022-09, Vol.47 (9), p.1694-1702 |
| issn | 0307-6938 1365-2230 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_2667788128 |
| source | Oxford University Press Journals All Titles (1996-Current); Alma/SFX Local Collection |
| subjects | Biopsy CD25 antigen CD30 antigen Children Dermis Localization Mast cells Mastocytoma Mastocytosis Patients Pediatrics Puberty Skin Skin cancer |
| title | Histopathological characteristics are instrumental to distinguish monomorphic from polymorphic maculopapular cutaneous mastocytosis in children |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-02-09T21%3A26%3A01IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-proquest_cross&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Histopathological%20characteristics%20are%20instrumental%20to%20distinguish%20monomorphic%20from%20polymorphic%20maculopapular%20cutaneous%20mastocytosis%20in%20children&rft.jtitle=Clinical%20and%20experimental%20dermatology&rft.au=Hermans,%20Maud%20A.%20W.&rft.date=2022-09&rft.volume=47&rft.issue=9&rft.spage=1694&rft.epage=1702&rft.pages=1694-1702&rft.issn=0307-6938&rft.eissn=1365-2230&rft_id=info:doi/10.1111/ced.15262&rft_dat=%3Cproquest_cross%3E2703902028%3C/proquest_cross%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=2703902028&rft_id=info:pmid/35596520&rfr_iscdi=true |