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KMID : 0363219950330030564
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Korean Journal of Dermatology
1995 Volume.33 No. 3 p.564 ~ p.569
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Three Cases of Hereditary Angiodema in One Family
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서광석
강정민/김계정/강형재
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Abstract
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hereditay angiodema is a rare genetic disease transmitted with an autosomal dominat trait result of a quantitative or functional defect of C1 inhibitor.
We report three of hereditary angioedema from the same family respectively the 35-year-old sister, the younger brother of 32, and the younger sister of 26. The older sister died due to her airway being obstructed by acute laryngeal edema, and
sister
experienced recurrent episodes of swellijg on the hand and forearm. Therefoue we examined the two patients' serum complement levels to confirm dragnosis. In both, the results showed decreased levels of C1 inhibitor and C4 which are the evidence
of
hereditary angioedema. The younger sister has been administered danazol (600mg/day) to prevent angioedema for 8 weeks, and we have not yet found a recurrence of the symptoms. However, we'll control the dosage of danazol because amenorrhea has
developed.
(Kor J Dermatol 1995;33(3) : 564~569)
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