METHYLTESTOSTERONE THERAPY FOR HEREDITARY EPISODIC EDEMA (HEREDITARY ANGIONEUROTIC EDEMA)

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Abstract
This rare disease is inherited as a mendelian dominant abnormality. The essential feature is a rapidly appearing, spreading edema which involves the skin or the wall of the mouth, pharynx, larynx or gastrointestinal tract. The attacks of edema usually occur at intervals of a few weeks or months, last several days and disappear completely leaving the patient well until the next attack. When the intestine is involved severe colicky abdominal pain occurs. Edema of the larynx may prove fatal. The usual name "hereditary angioneurotic edema" is misleading. Neurosis is not a feature of the disease nor is there proof that the fundamental disorder is a defect in the nervous control of blood vessels. The suggested name "hereditary episodic edema" emphasizes the recurrent character of the edema and removes the implications of the word "angioneurotic". The administration of methyltestosterone as linguets or tablets was found to prevent attacks of edema in 4 members of an affected family. In 3 cases attacks reappeared during the use of placebos. The daily dose was small and equivalent to that used in replacement therapy in conditions where the production of androgens is deficient.

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