Luiz Fernando Bacarini, Ana Luisa Vieira, Mariana Camargo, Tainá Mosca, Wilma Carvalho Neves-Forte
{"title":"【遗传性血管性水肿诊断三十年后临床表现】。","authors":"Luiz Fernando Bacarini, Ana Luisa Vieira, Mariana Camargo, Tainá Mosca, Wilma Carvalho Neves-Forte","doi":"10.29262/ram.v68i3.874","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Diagnosis and treatment of hereditary angioedema (HAE) are necessary to improve the quality of life and even the survival of patients.</p><p><strong>Case report: </strong>A 52-year-old woman with angioedema for 30 years, which affects the face, tongue, and hands. It is asymmetric, with neither pruritus nor urticaria, without response to antihistamines or corticosteroids, with spontaneous resolution in 48 hours to 72 hours; with a family history of angioedema. Normal physical examination between exacerbations. Autoimmune and lymphoproliferative diseases were ruled out. Values of C1q, C4, C1-INH were normal. The diagnosis of HAE type C1-INH normal subtype Unknown was established. The total resolution of the crises was achieved after two months with androgen therapy. Outpatient follow-up has been given for four years and no angioedema crisis has been reported, which is associated with a radical change in the quality of life.</p><p><strong>Conclusion: </strong>The patient was diagnosed with HAE after 30 years of clinical manifestations, after acquired angioedema was ruled out.</p>","PeriodicalId":21175,"journal":{"name":"Revista alergia Mexico","volume":"68 3","pages":"206-208"},"PeriodicalIF":0.0000,"publicationDate":"2021-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"[Diagnosis of hereditary angioedema after thirty years of clinical manifestations].\",\"authors\":\"Luiz Fernando Bacarini, Ana Luisa Vieira, Mariana Camargo, Tainá Mosca, Wilma Carvalho Neves-Forte\",\"doi\":\"10.29262/ram.v68i3.874\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>Diagnosis and treatment of hereditary angioedema (HAE) are necessary to improve the quality of life and even the survival of patients.</p><p><strong>Case report: </strong>A 52-year-old woman with angioedema for 30 years, which affects the face, tongue, and hands. It is asymmetric, with neither pruritus nor urticaria, without response to antihistamines or corticosteroids, with spontaneous resolution in 48 hours to 72 hours; with a family history of angioedema. Normal physical examination between exacerbations. Autoimmune and lymphoproliferative diseases were ruled out. Values of C1q, C4, C1-INH were normal. The diagnosis of HAE type C1-INH normal subtype Unknown was established. The total resolution of the crises was achieved after two months with androgen therapy. Outpatient follow-up has been given for four years and no angioedema crisis has been reported, which is associated with a radical change in the quality of life.</p><p><strong>Conclusion: </strong>The patient was diagnosed with HAE after 30 years of clinical manifestations, after acquired angioedema was ruled out.</p>\",\"PeriodicalId\":21175,\"journal\":{\"name\":\"Revista alergia Mexico\",\"volume\":\"68 3\",\"pages\":\"206-208\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2021-07-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Revista alergia Mexico\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.29262/ram.v68i3.874\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q3\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Revista alergia Mexico","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.29262/ram.v68i3.874","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"Medicine","Score":null,"Total":0}
[Diagnosis of hereditary angioedema after thirty years of clinical manifestations].
Introduction: Diagnosis and treatment of hereditary angioedema (HAE) are necessary to improve the quality of life and even the survival of patients.
Case report: A 52-year-old woman with angioedema for 30 years, which affects the face, tongue, and hands. It is asymmetric, with neither pruritus nor urticaria, without response to antihistamines or corticosteroids, with spontaneous resolution in 48 hours to 72 hours; with a family history of angioedema. Normal physical examination between exacerbations. Autoimmune and lymphoproliferative diseases were ruled out. Values of C1q, C4, C1-INH were normal. The diagnosis of HAE type C1-INH normal subtype Unknown was established. The total resolution of the crises was achieved after two months with androgen therapy. Outpatient follow-up has been given for four years and no angioedema crisis has been reported, which is associated with a radical change in the quality of life.
Conclusion: The patient was diagnosed with HAE after 30 years of clinical manifestations, after acquired angioedema was ruled out.