Maculopapular cutaneous Mastocytosis
Autor(a) principal: | |
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Data de Publicação: | 2022 |
Outros Autores: | , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | http://hdl.handle.net/10400.16/2766 |
Resumo: | Mastocytosis is a rare disorder caused by proliferation and accumulation of mast cells in various tissues, with characteristic symptoms associated with the release of their mediators. Its cutaneous form, usually benign, is the most frequent among children. The authors report the case of a two-month-old male infant who presented to the Emergency Department with small, tan-to-brown macules and papules distributed mainly on the trunk, which progressively became nodular and bullous, with one month of evolution. Darier´s sign was positive. The absence of systemic signs and analytic (including serum tryptase level) and echographic changes was consistent with the diagnosis of maculopapular cutaneous mastocytosis, or urticaria pigmentosa, subsequently confirmed by skin biopsy. Treatment with anti-histaminic therapy and topical immunosuppressant and avoidance of triggering factors led to a positive outcome. |
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Maculopapular cutaneous MastocytosisMastocitose cutânea maculopapularDariermastocitosePediatricsMastocytosis is a rare disorder caused by proliferation and accumulation of mast cells in various tissues, with characteristic symptoms associated with the release of their mediators. Its cutaneous form, usually benign, is the most frequent among children. The authors report the case of a two-month-old male infant who presented to the Emergency Department with small, tan-to-brown macules and papules distributed mainly on the trunk, which progressively became nodular and bullous, with one month of evolution. Darier´s sign was positive. The absence of systemic signs and analytic (including serum tryptase level) and echographic changes was consistent with the diagnosis of maculopapular cutaneous mastocytosis, or urticaria pigmentosa, subsequently confirmed by skin biopsy. Treatment with anti-histaminic therapy and topical immunosuppressant and avoidance of triggering factors led to a positive outcome.Centro Hospitalar Universitário do PortoRepositório Científico do Centro Hospitalar Universitário de Santo AntónioSoares Pinho, CrisbetyBarreto, InêsRebimbas, SandraCoelho, Sónia2023-02-02T11:01:13Z2022-032022-03-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10400.16/2766engNascer e Crescer - Birth and Growth Medical Journal 2022;31(1):68-72. doi:10.25753/BirthGrowthMJ.v31.i1.23848https://doi.org/10.25753/BirthGrowthMJ.v31.i1.238482183-9417info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-10-20T11:02:24Zoai:repositorio.chporto.pt:10400.16/2766Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T20:38:56.944107Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Maculopapular cutaneous Mastocytosis Mastocitose cutânea maculopapular |
title |
Maculopapular cutaneous Mastocytosis |
spellingShingle |
Maculopapular cutaneous Mastocytosis Soares Pinho, Crisbety Darier mastocitose Pediatrics |
title_short |
Maculopapular cutaneous Mastocytosis |
title_full |
Maculopapular cutaneous Mastocytosis |
title_fullStr |
Maculopapular cutaneous Mastocytosis |
title_full_unstemmed |
Maculopapular cutaneous Mastocytosis |
title_sort |
Maculopapular cutaneous Mastocytosis |
author |
Soares Pinho, Crisbety |
author_facet |
Soares Pinho, Crisbety Barreto, Inês Rebimbas, Sandra Coelho, Sónia |
author_role |
author |
author2 |
Barreto, Inês Rebimbas, Sandra Coelho, Sónia |
author2_role |
author author author |
dc.contributor.none.fl_str_mv |
Repositório Científico do Centro Hospitalar Universitário de Santo António |
dc.contributor.author.fl_str_mv |
Soares Pinho, Crisbety Barreto, Inês Rebimbas, Sandra Coelho, Sónia |
dc.subject.por.fl_str_mv |
Darier mastocitose Pediatrics |
topic |
Darier mastocitose Pediatrics |
description |
Mastocytosis is a rare disorder caused by proliferation and accumulation of mast cells in various tissues, with characteristic symptoms associated with the release of their mediators. Its cutaneous form, usually benign, is the most frequent among children. The authors report the case of a two-month-old male infant who presented to the Emergency Department with small, tan-to-brown macules and papules distributed mainly on the trunk, which progressively became nodular and bullous, with one month of evolution. Darier´s sign was positive. The absence of systemic signs and analytic (including serum tryptase level) and echographic changes was consistent with the diagnosis of maculopapular cutaneous mastocytosis, or urticaria pigmentosa, subsequently confirmed by skin biopsy. Treatment with anti-histaminic therapy and topical immunosuppressant and avoidance of triggering factors led to a positive outcome. |
publishDate |
2022 |
dc.date.none.fl_str_mv |
2022-03 2022-03-01T00:00:00Z 2023-02-02T11:01:13Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://hdl.handle.net/10400.16/2766 |
url |
http://hdl.handle.net/10400.16/2766 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
Nascer e Crescer - Birth and Growth Medical Journal 2022;31(1):68-72. doi:10.25753/BirthGrowthMJ.v31.i1.23848 https://doi.org/10.25753/BirthGrowthMJ.v31.i1.23848 2183-9417 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Centro Hospitalar Universitário do Porto |
publisher.none.fl_str_mv |
Centro Hospitalar Universitário do Porto |
dc.source.none.fl_str_mv |
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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