CUTANEOUS MASTOCYTOSIS IN CHILDHOOD – RETROSPECTIVE STUDY OF 32 PATIENTS
Autor(a) principal: | |
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Data de Publicação: | 2013 |
Outros Autores: | , , , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | https://doi.org/10.29021/spdv.70.4.99 |
Resumo: | Introduction: Mastocytosis is a broad term that includes a set of clinically distinct entities characterized by the accumulation of mast cells in tissues. The skin is the organ most frequently involved. There are four clinical pat- terns of cutaneous mastocytosis: urticaria pigmentosa (UP), diffuse cutaneous mastocytosis, mastocytoma and telan- giectasia macularis eruptiva perstans. In childhood, the disease is usually limited to the skin. Material and Methods: Retrospective study of patients with cutaneous mastocytosis of the Pediatric Dermatology Outpatient Clinic at Hospital de Curry Cabral between 2001 and 2010. Results: Thirty-two patients were included (20 male and 12 female). In 90.6% of the cases, the manifestations began before two years of age. Only urticaria pigmentosa (53.1%) and mastocytomas (46.9%) were observed. The trunk and proximal portion of the limbs were the preferred locations. Darier ́s sign was pre- sent in 87.5% of cases (94.1% of UP and 80% of mastocytomas). The associated manifestations were pruritus (40.6%), blistering (28.1%), flushing (18.8%) and dermographism (12.5%). There were no cases of systemic mastocytosis. Two patients had a family history of cutaneous mastocytosis. No significant laboratory abnormalities were detected. In the 16 cases determined, serum levels of alpha-tryptase were within normal range. Discussion: These results are consistent with the literature, particularly in relation to common clinical forms, the usual locations, high frequency of Darier ́s sign and early age of onset. Once determined, the serum levels of alpha-tryptase were normal, which is consistent with the absence of systemic involvement. The higher prevalence in males has been reported before.KEYWORDS – Mastocytosis, cutaneous; Child. |
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CUTANEOUS MASTOCYTOSIS IN CHILDHOOD – RETROSPECTIVE STUDY OF 32 PATIENTSMASTOCITOSE CUTÂNEA NA INFÂNCIA – ESTUDO RETROSPETIVO DE 32 DOENTESIntroduction: Mastocytosis is a broad term that includes a set of clinically distinct entities characterized by the accumulation of mast cells in tissues. The skin is the organ most frequently involved. There are four clinical pat- terns of cutaneous mastocytosis: urticaria pigmentosa (UP), diffuse cutaneous mastocytosis, mastocytoma and telan- giectasia macularis eruptiva perstans. In childhood, the disease is usually limited to the skin. Material and Methods: Retrospective study of patients with cutaneous mastocytosis of the Pediatric Dermatology Outpatient Clinic at Hospital de Curry Cabral between 2001 and 2010. Results: Thirty-two patients were included (20 male and 12 female). In 90.6% of the cases, the manifestations began before two years of age. Only urticaria pigmentosa (53.1%) and mastocytomas (46.9%) were observed. The trunk and proximal portion of the limbs were the preferred locations. Darier ́s sign was pre- sent in 87.5% of cases (94.1% of UP and 80% of mastocytomas). The associated manifestations were pruritus (40.6%), blistering (28.1%), flushing (18.8%) and dermographism (12.5%). There were no cases of systemic mastocytosis. Two patients had a family history of cutaneous mastocytosis. No significant laboratory abnormalities were detected. In the 16 cases determined, serum levels of alpha-tryptase were within normal range. Discussion: These results are consistent with the literature, particularly in relation to common clinical forms, the usual locations, high frequency of Darier ́s sign and early age of onset. Once determined, the serum levels of alpha-tryptase were normal, which is consistent with the absence of systemic involvement. The higher prevalence in males has been reported before.KEYWORDS – Mastocytosis, cutaneous; Child. Introdução: A designação de mastocitose engloba várias entidades clinicamente distintas caracterizadas pela acumulação tissular de mastócitos. A pele é o órgão mais frequentemente envolvido. Consideram-se 4 padrões clínicos de mastocitose cutânea: urticária pigmentosa (UP), mastocitose cutânea difusa, mastocitoma e telangiec- tasia macularis eruptiva perstans. Na infância, a doença é habitualmente autolimitada e exclusivamente cutânea. Material e Métodos: Apresenta-se um estudo retrospetivo dos doentes com mastocitose cutânea observados na Con- sulta de Dermatologia Pediátrica do Hospital de Curry Cabral entre 2001 e 2010. Resultados: Foram englobados 32 doentes (20 do sexo masculino e 12 do sexo feminino). Em 90.6% dos casos, as manifestações surgiram antes dos 2 anos. Apenas foram observadas UP (53,1%) e mastocitomas (46,9%). O tronco e a raíz dos membros foram as locali- zações preferenciais. O sinal de Darier estava presente em 87,5% dos casos (94,1% das UP e 80% dos mastocitomas). As manifestações associadas foram: prurido (40,6%), formação de bolha (28,1%), flushing (18,8%) e dermografismo (12,5%). Não foram documentados casos de mastocitose sistémica. Dois doentes tinham história familiar de mas- tocitose cutânea. Não foram detetadas alterações laboratoriais significativas. Nos 16 casos determinados, os níveis séricos de Triptase-alfa foram normais. Discussão: Estes resultados estão de acordo com a literatura, nomeadamente em relação às formas clínicas mais frequentes, localizações habituais, elevada frequência do sinal de Darier e idade precoce de início. Quando determinados, os níveis séricos de Triptase-alfa foram normais, o que está de acordo com a ausência de envolvimento sistémico. A maior prevalência no sexo masculino tem sido relatada em alguns estudos. PALAVRAS-CHAVE – Mastocitose cutânea; Criança. Sociedade Portuguesa de Dermatologia e Venereologia2013-04-20T00:00:00Zjournal articleinfo:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://doi.org/10.29021/spdv.70.4.99oai:ojs.revista.spdv.com.pt:article/99Journal of the Portuguese Society of Dermatology and Venereology; Vol 70 No 4 (2012): Outubro-Dezembro; 459-463Revista da Sociedade Portuguesa de Dermatologia e Venereologia; v. 70 n. 4 (2012): Outubro-Dezembro; 459-4632182-24092182-2395reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPporhttps://revista.spdv.com.pt/index.php/spdv/article/view/99https://doi.org/10.29021/spdv.70.4.99https://revista.spdv.com.pt/index.php/spdv/article/view/99/96Macias, Vasco CoelhoAmaro, CristinaFreitas, IsabelVieira, RaquelCardoso, Jorgeinfo:eu-repo/semantics/openAccess2022-10-06T12:34:40Zoai:ojs.revista.spdv.com.pt:article/99Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T16:10:41.360794Repositório 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 |
CUTANEOUS MASTOCYTOSIS IN CHILDHOOD – RETROSPECTIVE STUDY OF 32 PATIENTS MASTOCITOSE CUTÂNEA NA INFÂNCIA – ESTUDO RETROSPETIVO DE 32 DOENTES |
title |
CUTANEOUS MASTOCYTOSIS IN CHILDHOOD – RETROSPECTIVE STUDY OF 32 PATIENTS |
spellingShingle |
CUTANEOUS MASTOCYTOSIS IN CHILDHOOD – RETROSPECTIVE STUDY OF 32 PATIENTS Macias, Vasco Coelho |
title_short |
CUTANEOUS MASTOCYTOSIS IN CHILDHOOD – RETROSPECTIVE STUDY OF 32 PATIENTS |
title_full |
CUTANEOUS MASTOCYTOSIS IN CHILDHOOD – RETROSPECTIVE STUDY OF 32 PATIENTS |
title_fullStr |
CUTANEOUS MASTOCYTOSIS IN CHILDHOOD – RETROSPECTIVE STUDY OF 32 PATIENTS |
title_full_unstemmed |
CUTANEOUS MASTOCYTOSIS IN CHILDHOOD – RETROSPECTIVE STUDY OF 32 PATIENTS |
title_sort |
CUTANEOUS MASTOCYTOSIS IN CHILDHOOD – RETROSPECTIVE STUDY OF 32 PATIENTS |
author |
Macias, Vasco Coelho |
author_facet |
Macias, Vasco Coelho Amaro, Cristina Freitas, Isabel Vieira, Raquel Cardoso, Jorge |
author_role |
author |
author2 |
Amaro, Cristina Freitas, Isabel Vieira, Raquel Cardoso, Jorge |
author2_role |
author author author author |
dc.contributor.author.fl_str_mv |
Macias, Vasco Coelho Amaro, Cristina Freitas, Isabel Vieira, Raquel Cardoso, Jorge |
description |
Introduction: Mastocytosis is a broad term that includes a set of clinically distinct entities characterized by the accumulation of mast cells in tissues. The skin is the organ most frequently involved. There are four clinical pat- terns of cutaneous mastocytosis: urticaria pigmentosa (UP), diffuse cutaneous mastocytosis, mastocytoma and telan- giectasia macularis eruptiva perstans. In childhood, the disease is usually limited to the skin. Material and Methods: Retrospective study of patients with cutaneous mastocytosis of the Pediatric Dermatology Outpatient Clinic at Hospital de Curry Cabral between 2001 and 2010. Results: Thirty-two patients were included (20 male and 12 female). In 90.6% of the cases, the manifestations began before two years of age. Only urticaria pigmentosa (53.1%) and mastocytomas (46.9%) were observed. The trunk and proximal portion of the limbs were the preferred locations. Darier ́s sign was pre- sent in 87.5% of cases (94.1% of UP and 80% of mastocytomas). The associated manifestations were pruritus (40.6%), blistering (28.1%), flushing (18.8%) and dermographism (12.5%). There were no cases of systemic mastocytosis. Two patients had a family history of cutaneous mastocytosis. No significant laboratory abnormalities were detected. In the 16 cases determined, serum levels of alpha-tryptase were within normal range. Discussion: These results are consistent with the literature, particularly in relation to common clinical forms, the usual locations, high frequency of Darier ́s sign and early age of onset. Once determined, the serum levels of alpha-tryptase were normal, which is consistent with the absence of systemic involvement. The higher prevalence in males has been reported before.KEYWORDS – Mastocytosis, cutaneous; Child. |
publishDate |
2013 |
dc.date.none.fl_str_mv |
2013-04-20T00:00:00Z |
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journal article info:eu-repo/semantics/article |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
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article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://doi.org/10.29021/spdv.70.4.99 oai:ojs.revista.spdv.com.pt:article/99 |
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https://doi.org/10.29021/spdv.70.4.99 |
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oai:ojs.revista.spdv.com.pt:article/99 |
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https://revista.spdv.com.pt/index.php/spdv/article/view/99 https://doi.org/10.29021/spdv.70.4.99 https://revista.spdv.com.pt/index.php/spdv/article/view/99/96 |
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info:eu-repo/semantics/openAccess |
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openAccess |
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Sociedade Portuguesa de Dermatologia e Venereologia |
publisher.none.fl_str_mv |
Sociedade Portuguesa de Dermatologia e Venereologia |
dc.source.none.fl_str_mv |
Journal of the Portuguese Society of Dermatology and Venereology; Vol 70 No 4 (2012): Outubro-Dezembro; 459-463 Revista da Sociedade Portuguesa de Dermatologia e Venereologia; v. 70 n. 4 (2012): Outubro-Dezembro; 459-463 2182-2409 2182-2395 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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