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.
Erythema Marginatum as an Early Symptom of Hereditary Angioedema: Case Report of 2 Newborns
POTENTIAL CONFLICT OF INTEREST: Dr Martinez-Saguer has received honoraria for lectures and funding for travel expenses from CSL Behring. She is a consultant for the following companies: Baxter, Bayer, CSL Behring, Octapharma, Shire, and Sobi. Dr Farkas has received consultancy/speaker fees and honoraria from Shire Human Genetic Therapies Inc., Swedish Orphan Biovitrum, and CSL Behring.
FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose.
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Inmaculada Martinez-Saguer, Henriette Farkas; Erythema Marginatum as an Early Symptom of Hereditary Angioedema: Case Report of 2 Newborns. Pediatrics February 2016; 137 (2): e20152411. 10.1542/peds.2015-2411
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