ABSTRACT
Angioedema is a potentially life threatening condition and may be either inherited or acquired. The latter is rare with only a handful of cases reported in the world literature. Presenting complaints are often vague. Those most commonly described include swelling in the subcutaneous and submucosal tissues. Patients presenting with laryngeal edema have high mortality, and high clinical suspicion is necessary to avoid instrumentation, which can precipitate laryngeal spasm. We present a review of reported cases of hormonally induced hereditary angioedema, along with a report of a patient with acquired angioedema secondary to hormone replacement therapy. To the best of our knowledge, this case probably represents the first reported case of acquired angioedema secondary to hormone replacement therapy.
Subject(s)
Androgens/therapeutic use , Angioedema/chemically induced , Dysmenorrhea/drug therapy , Female , Hormone Replacement Therapy/adverse effects , Humans , Middle Aged , Stanozolol/therapeutic useABSTRACT
Se realiza una revisión del tema edema angioneurótico hereditario. Después de una introducción histórica se considera el sistema del complemento, el sistema de las cininas, los inhibidores de las proteasas hemáticas derivadas del plasminógeno y la vinculación entre ambos. Se consideran también los avances últimos en lo que a citogenética molecular se refiere. Se presentan las diferentes formas clínicas de los edemas angioneuróticos hereditarios, así como sus diferencias clínico-laboratoriales. Se hace una valoración de los tratamientos sustitutivos y profilácticos. Se expone el estudio de tres casos clínicos observados en el lapso de 30 años de práctica dermatológica.
Subject(s)
Humans , Male , Female , Adult , Angioedema/genetics , /administration & dosage , /therapeutic use , Androgens/therapeutic use , Angioedema/complications , Angioedema/physiopathology , Kallikreins/adverse effects , Kallikreins/physiology , Cinnarizine/administration & dosage , Cinnarizine/therapeutic use , Complement C1s , Complement C1s/deficiency , Complement System Proteins , Diagnosis, Differential , Genetic Code , Kinins/adverse effects , Plasma , Prognosis , Stanozolol/administration & dosage , Stanozolol/therapeutic use , Suramin/administration & dosage , Suramin/therapeutic useSubject(s)
Humans , Angioedema/immunology , Autoimmunity/immunology , Complement C1 Inactivator Proteins/deficiency , Androgens , Angioedema/diagnosis , Angioedema/physiopathology , Complement C1 Inactivator Proteins/adverse effects , Complement C1 Inactivator Proteins/physiology , Danazol , Diagnosis, Differential , Laryngeal Edema/complications , Genetic Diseases, Inborn , Recurrence , Skin Manifestations , Stanozolol/therapeutic useABSTRACT
O angioedema herditário, forma congênita, é uma doença rara autossômica dominante, caracterizada por apresentar episódios de angiodema, localizados ou generalizados, potencialmente fatais, decorrentes da deficiência quantitativa e/ou qualitativa do inibidor de C1-esterase, induzindo a reduçäo ou ausência das fraçöes do complemento sérico C2, C4 e CH50. Apresentamos e discutimos os casos de dois irmäos portadores desta afecçäo, controlados adequadamente (quadros clínicos e imunoquímico) com o androgêno estanazol