Medical therapy for the syndrome of familial virilization, insulin resistance, and acanthosis nigricans.
In the syndrome of familial virilization, insulin resistance, and acanthosis nigricans, the interrelationships are not understood. Twin sisters were studied, along with a lesser affected sister and mother. They manifested amenorrhea, hirsutism, masculinization, hypertension, hyperinsulinemia, hypertriglyceridemia, and hyperprolactinemia. Medical therapy with a gonadotropin-releasing hormone agonist plus an antiandrogen resulted in reversal of the hirsutism, yet with preservation of potential fertility. In response to luteinizing hormone (LH) and follicle-stimulating hormone suppression, there was normalization of the serum androgens, but not of the hyperinsulinemia, hypertriglyceridemia, hyperprolactinemia, hypertension, or acanthosis nigricans.


CONCLUSIONS
(1) This syndrome may be familial. (2) Medical therapy for the virilization is successful. (3) The hyperandrogenemia is primarily LH dependent and not primarily insulin dependent, although insulin may have an amplification effect. (4) Hyperinsulinemia, hypertriglyceridemia, hyperprolactinemia, and the hypertension are not androgen dependent.
Corenblum
B
B
Department of Internal Medicine, University of Calgary, Alberta, Canada.
Baylis
B W
BW
D000052:Acanthosis Nigricans / Q000188:drug therapy* / Q000235:genetics / Q000378:metabolism; D000328:Adult; D000726:Androgen Antagonists / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D003534:Cyproterone / Q000031:analogs & derivatives / Q000627:therapeutic use; D017373:Cyproterone Acetate; D003907:Dexamethasone / Q000627:therapeutic use; D004200:Diseases in Twins / Q000235:genetics / Q000628:therapy; D005192:Family Health; D005260:Female; D005640:Follicle Stimulating Hormone / Q000097:blood; D007987:Gonadotropin-Releasing Hormone / Q000031:analogs & derivatives / Q000627:therapeutic use; D006628:Hirsutism / Q000188:drug therapy / Q000235:genetics / Q000378:metabolism; D006801:Humans; D006946:Hyperinsulinism / Q000188:drug therapy / Q000378:metabolism; D006949:Hyperlipidemias / Q000188:drug therapy / Q000378:metabolism; D006966:Hyperprolactinemia / Q000188:drug therapy / Q000378:metabolism; D006973:Hypertension / Q000188:drug therapy / Q000378:metabolism; D007333:Insulin Resistance / Q000235:genetics*; D016729:Leuprolide; D007986:Luteinizing Hormone / Q000097:blood; D008297:Male; D010906:Pituitary Hormone-Releasing Hormones / Q000502:physiology; D013577:Syndrome; D014770:Virilism / Q000188:drug therapy* / Q000235:genetics / Q000378:metabolism
D000726:Androgen Antagonists; D000970:Antineoplastic Agents; D010906:Pituitary Hormone-Releasing Hormones; D007987:Gonadotropin-Releasing Hormone; D017373:Cyproterone Acetate; D003907:Dexamethasone; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone; D003534:Cyproterone; D016729:Leuprolide