The care pathway for children with urticaria, angioedema, mastocytosis
| Autor: | Giovanni Passalacqua, Giovanni Corsello, Stefania La Grutta, Valeria Scavone, Giuliana Ferrante, Emilia Adrignola, Maria Concetta Muscia |
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| Přispěvatelé: | Ferrante, G., Scavone, V., Muscia, M., Adrignola, E., Corsello, G., Passalacqua, G., La Grutta, S. |
| Rok vydání: | 2015 |
| Předmět: |
lcsh:Immunologic diseases. Allergy
Pulmonary and Respiratory Medicine Allergy Urticaria Epidemiology Immunology Review Clinical practice Angioedema Children Diagnosis Itch Management Mastocytosis Skin C1-inhibitor 030207 dermatology & venereal diseases 03 medical and health sciences 0302 clinical medicine immune system diseases Edema medicine Immunology and Allergy skin and connective tissue diseases biology business.industry Autosomal dominant trait Angioneurotic oedema medicine.disease Pathophysiology 3. Good health Urticaria Angioedema Mastocytosis Skin Itch Children Epidemiology Diagnosis Management Clinical practice 030228 respiratory system Hereditary angioedema biology.protein medicine.symptom lcsh:RC581-607 business |
| Zdroj: | The World Allergy Organization Journal World Allergy Organization Journal, Vol 8, Iss, Pp-(2015) |
| ISSN: | 1939-4551 |
| DOI: | 10.1186/s40413-014-0052-x |
| Popis: | 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. Keywords: Urticaria, Angioedema, Mastocytosis, Skin, Itch, Children, Epidemiology, Diagnosis, Management, Clinical practice |
| Databáze: | OpenAIRE |
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