Male Pseudohermaphroditism Often Results From Resistance to Androgens

A pseudohermaphrodite is an individual with the gonads of one sex and the genitalia of the other. One of the most interesting causes of male reproductive abnormalities is an end organ insensitivity to androgens. The best characterized syndrome is testicular feminization, an X-linked recessive disorder caused by a defect in the testosterone receptor. In the classical form, patients are male pseudohermaphrodites with a female phenotype and an XY male genotype. They have abdominal testes that secrete testosterone but no other internal genitalia of either sex (see Chapter 39). They commonly have female external genitalia, but with a short vagina ending in a blind pouch. Breast development is typical of a female (as a result of peripheral aromatization of testosterone), but axillary and pubic hair, which are androgen-dependent, are scarce or absent. Testosterone levels are normal or elevated, estradiol levels are above the normal male range, and circulating go-nadotropin levels are high. The inguinally located testes usually have to be removed because of an increased risk of cancer. After orchiectomy, patients are treated with estradiol to maintain a female phenotype.

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