Pediatric Angioedema without Wheals: How to Guide the Diagnosis

Angioedema (AE) is a vascular reaction of subcutaneous and submucosal tissues that identifies various clinical pictures and often is associated with wheals. AE without wheals (AEwW) is infrequent. The ability to distinguish between AEwW mediated by mast cells and bradykinin-mediated or leukotriene-m...

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Main Authors: Lucia Liotti, Luca Pecoraro, Carla Mastrorilli, Riccardo Castagnoli, Francesca Saretta, Francesca Mori, Stefania Arasi, Simona Barni, Mattia Giovannini, Lucia Caminiti, Michele Miraglia Del Giudice, Elio Novembre
Format: Article
Language:English
Published: MDPI AG 2023-04-01
Series:Life
Subjects:
Online Access:https://www.mdpi.com/2075-1729/13/4/1021
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author Lucia Liotti
Luca Pecoraro
Carla Mastrorilli
Riccardo Castagnoli
Francesca Saretta
Francesca Mori
Stefania Arasi
Simona Barni
Mattia Giovannini
Lucia Caminiti
Michele Miraglia Del Giudice
Elio Novembre
author_facet Lucia Liotti
Luca Pecoraro
Carla Mastrorilli
Riccardo Castagnoli
Francesca Saretta
Francesca Mori
Stefania Arasi
Simona Barni
Mattia Giovannini
Lucia Caminiti
Michele Miraglia Del Giudice
Elio Novembre
author_sort Lucia Liotti
collection DOAJ
description Angioedema (AE) is a vascular reaction of subcutaneous and submucosal tissues that identifies various clinical pictures and often is associated with wheals. AE without wheals (AEwW) is infrequent. The ability to distinguish between AEwW mediated by mast cells and bradykinin-mediated or leukotriene-mediated pathways is often crucial for a correct diagnostic–therapeutic and follow-up approach. AEwW can be hereditary or acquired. Factors typically correlated with hereditary angioedema (HAE) are a recurrence of episodes, familiarity, association with abdominal pain, onset after trauma or invasive procedures, refractoriness to antiallergic therapy, and lack of pruritus. The acquired forms of AE can present a definite cause based on the anamnesis and diagnostic tests. Still, they can also have an undetermined cause (idiopathic AE), distinguished according to the response to antihistamine in histamine-mediated and non-histamine-mediated forms. Usually, in childhood, AE responds to antihistamines. If AEwW is not responsive to commonly used treatments, it is necessary to consider alternative diagnoses, even for pediatric patients. In general, a correct diagnostic classification allows, in most cases, optimal management of the patient with the prescription of appropriate therapy and the planning of an adequate follow-up.
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spelling doaj.art-ec979d6b621e40a4b8246e3a8609f1ca2023-11-17T20:07:02ZengMDPI AGLife2075-17292023-04-01134102110.3390/life13041021Pediatric Angioedema without Wheals: How to Guide the DiagnosisLucia Liotti0Luca Pecoraro1Carla Mastrorilli2Riccardo Castagnoli3Francesca Saretta4Francesca Mori5Stefania Arasi6Simona Barni7Mattia Giovannini8Lucia Caminiti9Michele Miraglia Del Giudice10Elio Novembre11Pediatric Unit, Department of Mother and Child Health, Salesi Children’s Hospital, 60123 Ancona, ItalyPediatric Unit, Department of Surgical Sciences, Dentistry, Gynecology and Pediatrics, University of Verona, 37126 Verona, ItalyPediatric Hospital Giovanni XXIII, Pediatric and Emergency Department, AOU Policlinic of Bari, 70126 Bari, ItalyDepartment of Clinical, Surgical, Diagnostic and Pediatric Sciences, University of Pavia, 27100 Pavia, ItalyPediatric Department, Latisana-Palmanova Hospital, Azienda Sanitaria Universitaria Friuli Centrale, 33100 Udine, ItalyAllergy Unit, Meyer Children’s Hospital IRCCS, 50139 Florence, ItalyTranslational Research in Pediatric Specialties Area, Division of Allergy, Bambino Gesù Children’s Hospital [IRCCS], 00165 Rome, ItalyAllergy Unit, Meyer Children’s Hospital IRCCS, 50139 Florence, ItalyAllergy Unit, Meyer Children’s Hospital IRCCS, 50139 Florence, ItalyDepartment of Human Pathology in Adult and Development Age “Gaetano Barresi”, Allergy Unit, Department of Pediatrics, AOU Policlinico Gaetano Martino, 98124 Messina, ItalyDepartment of Woman, Child and General and Specialized Surgery, University of Campania “Luigi Vanvitelli”, 80138 Naples, ItalyDepartment of Health Sciences, University of Florence, 50139 Florence, ItalyAngioedema (AE) is a vascular reaction of subcutaneous and submucosal tissues that identifies various clinical pictures and often is associated with wheals. AE without wheals (AEwW) is infrequent. The ability to distinguish between AEwW mediated by mast cells and bradykinin-mediated or leukotriene-mediated pathways is often crucial for a correct diagnostic–therapeutic and follow-up approach. AEwW can be hereditary or acquired. Factors typically correlated with hereditary angioedema (HAE) are a recurrence of episodes, familiarity, association with abdominal pain, onset after trauma or invasive procedures, refractoriness to antiallergic therapy, and lack of pruritus. The acquired forms of AE can present a definite cause based on the anamnesis and diagnostic tests. Still, they can also have an undetermined cause (idiopathic AE), distinguished according to the response to antihistamine in histamine-mediated and non-histamine-mediated forms. Usually, in childhood, AE responds to antihistamines. If AEwW is not responsive to commonly used treatments, it is necessary to consider alternative diagnoses, even for pediatric patients. In general, a correct diagnostic classification allows, in most cases, optimal management of the patient with the prescription of appropriate therapy and the planning of an adequate follow-up.https://www.mdpi.com/2075-1729/13/4/1021angioedemaidiopathic angioedemahereditary angioedemahistaminebradykinin
spellingShingle Lucia Liotti
Luca Pecoraro
Carla Mastrorilli
Riccardo Castagnoli
Francesca Saretta
Francesca Mori
Stefania Arasi
Simona Barni
Mattia Giovannini
Lucia Caminiti
Michele Miraglia Del Giudice
Elio Novembre
Pediatric Angioedema without Wheals: How to Guide the Diagnosis
Life
angioedema
idiopathic angioedema
hereditary angioedema
histamine
bradykinin
title Pediatric Angioedema without Wheals: How to Guide the Diagnosis
title_full Pediatric Angioedema without Wheals: How to Guide the Diagnosis
title_fullStr Pediatric Angioedema without Wheals: How to Guide the Diagnosis
title_full_unstemmed Pediatric Angioedema without Wheals: How to Guide the Diagnosis
title_short Pediatric Angioedema without Wheals: How to Guide the Diagnosis
title_sort pediatric angioedema without wheals how to guide the diagnosis
topic angioedema
idiopathic angioedema
hereditary angioedema
histamine
bradykinin
url https://www.mdpi.com/2075-1729/13/4/1021
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