{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,28]],"date-time":"2026-10-28T22:39:45Z","timestamp":1793227185431,"version":"4.5.0"},"reference-count":23,"publisher":"American Academy of Pediatrics (AAP)","issue":"2","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2016,2,1]]},"abstract":"<jats:p>Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is a rare genetic disease that causes recurrent swelling attacks that may affect various body tissues. Angioedematous attacks can be fatal in the case of upper airway edema and are often preceded by prodromal symptoms like erythema marginatum. Initial symptoms usually occur in the first decade of life. We report on manifestation of profound and recurrent erythema marginatum in 2 newborns. In both cases, prodromal symptoms could help determine the diagnosis of C1-INH-HAE such that, at a later time, angioedematous attacks could be treated promptly and effectively. Awareness of C1-INH-HAE is low among physicians and even lower among the general public. This report aims at raising the level of awareness and shows that initial symptoms of the potentially life-threatening condition can manifest in newborns and that erythema marginatum can even be present at birth. Recognition of early symptoms and timely diagnosis of the disease along with adequate education of the pediatrician and parents are a prerequisite for prompt and effective treatment of attacks and the successful management of the disease.<\/jats:p>","DOI":"10.1542\/peds.2015-2411","type":"journal-article","created":{"date-parts":[[2016,1,13]],"date-time":"2016-01-13T07:56:09Z","timestamp":1452671769000},"source":"Crossref","is-referenced-by-count":43,"title":["Erythema Marginatum as an Early Symptom of Hereditary Angioedema: Case Report of 2 Newborns"],"prefix":"10.1542","volume":"137","author":[{"given":"Inmaculada","family":"Martinez-Saguer","sequence":"first","affiliation":[{"name":"Hemophilia Center Rhine Main GmbH, Germany; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henriette","family":"Farkas","sequence":"additional","affiliation":[{"name":"Hungarian Angioedema Center, 3rd Department of Internal Medicine, Semmelweis University, Hungary"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"417","published-online":{"date-parts":[[2016,2,1]]},"reference":[{"key":"2021120907174009900_B1","doi-asserted-by":"crossref","first-page":"S51","DOI":"10.1016\/j.jaci.2004.06.047","article-title":"Hereditary and acquired angioedema: problems and progress: proceedings of the third C1 esterase inhibitor deficiency workshop and beyond.","volume":"114","author":"Agostoni","year":"2004","journal-title":"J Allergy Clin Immunol"},{"issue":"3","key":"2021120907174009900_B2","doi-asserted-by":"crossref","first-page":"261","DOI":"10.2500\/aap.2013.34.3663","article-title":"Signs and symptoms preceding acute attacks of hereditary angioedema: results of three recent surveys.","volume":"34","author":"Reshef","year":"2013","journal-title":"Allergy Asthma Proc"},{"key":"2021120907174009900_B3","author":"Magerl","year":"2014"},{"issue":"4","key":"2021120907174009900_B4","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1097\/00000441-188804000-00004","article-title":"Hereditary angio-neurotic oedema.","volume":"95","author":"Osler","year":"1888","journal-title":"Am J Med Sci"},{"issue":"5","key":"2021120907174009900_B5","doi-asserted-by":"crossref","first-page":"506","DOI":"10.2500\/aap.2009.30.3279","article-title":"Frequency, timing, and type of prodromal symptoms associated with hereditary angioedema attacks.","volume":"30","author":"Prematta","year":"2009","journal-title":"Allergy Asthma Proc"},{"issue":"5","key":"2021120907174009900_B6","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1080\/000155501317140188","article-title":"Erythema marginatum preceding an acute oedematous attack of hereditary angioneurotic oedema.","volume":"81","author":"Farkas","year":"2001","journal-title":"Acta Derm Venereol"},{"issue":"6","key":"2021120907174009900_B7","doi-asserted-by":"crossref","first-page":"352","DOI":"10.1016\/0091-6749(74)90119-5","article-title":"Erythema marginatum preceding hereditary angioedema.","volume":"53","author":"Starr","year":"1974","journal-title":"J Allergy Clin Immunol"},{"issue":"5","key":"2021120907174009900_B8","doi-asserted-by":"crossref","first-page":"1153","DOI":"10.1111\/j.1365-2133.2009.09366.x","article-title":"Hereditary angio-oedema in Denmark: a nationwide survey.","volume":"161","author":"Bygum","year":"2009","journal-title":"Br J Dermatol"},{"issue":"3","key":"2021120907174009900_B9","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1016\/S0151-9638(05)79255-3","article-title":"Reticular erythema signalling the onset of episodes of hereditary angioedema in a child [in French]","volume":"132","author":"Hubiche","year":"2005","journal-title":"Ann Dermatol Venereol"},{"issue":"1","key":"2021120907174009900_B10","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1111\/j.1525-1470.2011.01675.x","article-title":"Hereditary angioedema in childhood: a challenging diagnosis you cannot afford to miss.","volume":"30","author":"Kjaer","year":"2013","journal-title":"Pediatr Dermatol"},{"issue":"3","key":"2021120907174009900_B11","doi-asserted-by":"crossref","first-page":"692","DOI":"10.1016\/j.jaci.2012.05.055","article-title":"Fatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency.","volume":"130","author":"Bork","year":"2012","journal-title":"J Allergy Clin Immunol"},{"issue":"3","key":"2021120907174009900_B12","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1111\/j.1572-0241.2006.00492.x","article-title":"Symptoms, course, and complications of abdominal attacks in hereditary angioedema due to C1 inhibitor deficiency.","volume":"101","author":"Bork","year":"2006","journal-title":"Am J Gastroenterol"},{"issue":"10","key":"2021120907174009900_B13","doi-asserted-by":"crossref","first-page":"1027","DOI":"10.1056\/NEJMcp0803977","article-title":"Clinical practice. 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