Three Cases of Hereditary Angioedema in One Family / 대한피부과학회지

ABSTRACT

Hereditary angioedema is a rare genetic disease transmitted with a autosomal dominant trait result of a quantitative or functional defect of Cl inhibitor. We report three cases of heriditary angioedema from the same famly respectively the 35-year-old sister, the younger bn)ther of 32, and the younger sister of 20 The older sister died due to her airway being obstructel by acute laryngeal edema, and her you igirbrother and sister experienced recurrent episodes oswelling on the hand and forearm. The ore we examined the two patients serum complement levils to confirm diagnosis. In both, the r;ults showed decreased levels of Cl inhibitor and C4 which are the evidence of hereditary a giedema. The younger sister has been administered danazol (600mg/day) to prevent angioedem or 8 weeks, and we have not yet found a recurrenc of he symptoms. However, well cont ol the dosage of danazol because amenorchea has developec.