ABSTRACT
Hereditary (HAE) and acquired (AAE) angioedema are vascular reactions involving the sub mucosal tissues, representing localized edema caused by dilatation and increased permeability of the capillaries. HAE and AAE are clinical disorders characterized by angioedema that require prompt differentiation from other causes of angioedema in order to receive the most pertinent and effective therapeutic interventions. The aim of this report is to describe the clinical characteristics of patients with both HAE and AAE identified and followed at the Immunology Clinic of the University Hospital at the Puerto Rico Medical Center, their response and side effects to danazol therapy and their comparison with other series of similar patients reported in the literature. Overall, the patients in this sample presented a similar clinical profile compared to other reported series in the literature.
Subject(s)
Humans , Male , Female , Child , Adolescent , Adult , Middle Aged , Angioedema , Estrogen Antagonists/administration & dosage , Estrogen Antagonists/adverse effects , Estrogen Antagonists/therapeutic use , Complement C1 , Complement C1q , Complement C4 , Data Interpretation, Statistical , Diagnosis, Differential , Danazol/administration & dosage , Danazol/adverse effects , Danazol/therapeutic use , Enzyme-Linked Immunosorbent Assay , Immunodiffusion , Complement Inactivating Agents/analysis , Time FactorsABSTRACT
Se presenta una paciente de 33 anos con episodios subitos de edema facial y de miembros superiories desencadenados por traumatismos leves. Los episodios ceden espontaneamente y no son prevenidos por la administracion de corticoides o antihistaminicos. El nivel de C1 inh fue de 40% y el de C4 se encontro disminuido, indicando consumo de los factores de la via clasica. Se analizan las caracteristicas clinicas y de laboratorio, concluyendo que el cuadro corresponde a angioedema hereditario causado por deficiencia de C1 inh.