Abstract

AMARAL, Luís; COIMBRA, Alice  and  PLACIDO, José Luís. Angioedema associado a ganglioneuroma: A propósito de um caso clínico. Rev Port Imunoalergologia [online]. 2015, vol.23, n.2, pp.89-93. ISSN 0871-9721.

Acquired angioedema is usually related with lymphoproliferative disorders, autoimmune diseases and adenocarcinomas but it is rarely associated with other forms of neoplasia. We report the case of an eight year old girl with recurrent episodes of unilateral labial and palpebral facial angioedema, without urticaria or pruritus that did not respond to oral corticosteroids (OC) and antihistamines (AH). No family history of angioedema nor trigger was identified. In may 2012, she went to the ER for se vere abdominal pain and ultrasound demonstrated the presence of a mediastinal mass. She was referred to Pediatric Oncology and admitted for excisional biopsy of the mass which permitted the diagnosis of ganglioneuroma. Total excision of the paraspinal mass was successful. No further episodes of angioedema were reported. In this article, we report the case of a child with recurrent angioedema, emphasizing the difficulties and the importance of a thorough clinical investigation in the clarification of angioedema etiology

Keywords : Acquired angioedema; C1 inhibitor deficiency; ganglioneuroma.

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