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The care pathway for children with urticaria, angioedema, mastocytosis

Cutaneous involvement characterized by urticarial lesions with or without angioedema and itch is commonly observed in routine medical practice. The clinical approach may still remain complex in real life, because several diseases may display similar cutaneous manifestations. Urticaria is a common di...

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Autores principales: Ferrante, Giuliana, Scavone, Valeria, Muscia, Maria Concetta, Adrignola, Emilia, Corsello, Giovanni, Passalacqua, Giovanni, La Grutta, Stefania
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4313464/
https://www.ncbi.nlm.nih.gov/pubmed/25674297
http://dx.doi.org/10.1186/s40413-014-0052-x
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author Ferrante, Giuliana
Scavone, Valeria
Muscia, Maria Concetta
Adrignola, Emilia
Corsello, Giovanni
Passalacqua, Giovanni
La Grutta, Stefania
author_facet Ferrante, Giuliana
Scavone, Valeria
Muscia, Maria Concetta
Adrignola, Emilia
Corsello, Giovanni
Passalacqua, Giovanni
La Grutta, Stefania
author_sort Ferrante, Giuliana
collection PubMed
description Cutaneous involvement characterized by urticarial lesions with or without angioedema and itch is commonly observed in routine medical practice. The clinical approach may still remain complex in real life, because several diseases may display similar cutaneous manifestations. Urticaria is a common disease, characterized by the sudden appearance of wheals, with/without angioedema. The term Chronic Urticaria (CU) encompasses a group of conditions with different underlying causes and different mechanisms, but sharing the clinical picture of recurring wheals and/or angioedema for at least 6 weeks. Hereditary Angioedema (HAE) is a rare disorder characterized by recurrent episodes of non-pruritic, non-pitting, subcutaneous or submucosal edema affecting the extremities, face, throat, trunk, genitalia, or bowel, that are referred as “attacks”. HAE is an autosomal dominant disease caused by a deficiency of functional C1 inhibitor, due to a mutation in C1-INH gene (serping 1 gene) characterized by the clonal proliferation of mast cells, leading to their accumulation, and possibly mediator release, in one or more organs. In childhood there are two main forms of mastocytosis, the Systemic and the Cutaneous. The clinical features of skin lesions in urticaria, angioedema and mastocytosis may differ depending on the aetiologic factors, and the underlying pathophysiological mechanisms. The diagnostic process, as stepwise approach in routine clinical practice, is here reviewed for CU, HAE and mastocytosis, resulting in an integrated method for improved management of these cutaneous diseases. Taking into account that usually these conditions have also a relevant impact on the quality of life of children, affecting social activities and behavior, the availability of care pathways could be helpful in disentangle the diagnostic issue achieving the most cost-effective ratio.
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spelling pubmed-43134642015-02-11 The care pathway for children with urticaria, angioedema, mastocytosis Ferrante, Giuliana Scavone, Valeria Muscia, Maria Concetta Adrignola, Emilia Corsello, Giovanni Passalacqua, Giovanni La Grutta, Stefania World Allergy Organ J Review Cutaneous involvement characterized by urticarial lesions with or without angioedema and itch is commonly observed in routine medical practice. The clinical approach may still remain complex in real life, because several diseases may display similar cutaneous manifestations. Urticaria is a common disease, characterized by the sudden appearance of wheals, with/without angioedema. The term Chronic Urticaria (CU) encompasses a group of conditions with different underlying causes and different mechanisms, but sharing the clinical picture of recurring wheals and/or angioedema for at least 6 weeks. Hereditary Angioedema (HAE) is a rare disorder characterized by recurrent episodes of non-pruritic, non-pitting, subcutaneous or submucosal edema affecting the extremities, face, throat, trunk, genitalia, or bowel, that are referred as “attacks”. HAE is an autosomal dominant disease caused by a deficiency of functional C1 inhibitor, due to a mutation in C1-INH gene (serping 1 gene) characterized by the clonal proliferation of mast cells, leading to their accumulation, and possibly mediator release, in one or more organs. In childhood there are two main forms of mastocytosis, the Systemic and the Cutaneous. The clinical features of skin lesions in urticaria, angioedema and mastocytosis may differ depending on the aetiologic factors, and the underlying pathophysiological mechanisms. The diagnostic process, as stepwise approach in routine clinical practice, is here reviewed for CU, HAE and mastocytosis, resulting in an integrated method for improved management of these cutaneous diseases. Taking into account that usually these conditions have also a relevant impact on the quality of life of children, affecting social activities and behavior, the availability of care pathways could be helpful in disentangle the diagnostic issue achieving the most cost-effective ratio. BioMed Central 2015-02-02 /pmc/articles/PMC4313464/ /pubmed/25674297 http://dx.doi.org/10.1186/s40413-014-0052-x Text en © Ferrante et al.; licensee BioMed Central. 2015 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Review
Ferrante, Giuliana
Scavone, Valeria
Muscia, Maria Concetta
Adrignola, Emilia
Corsello, Giovanni
Passalacqua, Giovanni
La Grutta, Stefania
The care pathway for children with urticaria, angioedema, mastocytosis
title The care pathway for children with urticaria, angioedema, mastocytosis
title_full The care pathway for children with urticaria, angioedema, mastocytosis
title_fullStr The care pathway for children with urticaria, angioedema, mastocytosis
title_full_unstemmed The care pathway for children with urticaria, angioedema, mastocytosis
title_short The care pathway for children with urticaria, angioedema, mastocytosis
title_sort care pathway for children with urticaria, angioedema, mastocytosis
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4313464/
https://www.ncbi.nlm.nih.gov/pubmed/25674297
http://dx.doi.org/10.1186/s40413-014-0052-x
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