Introduction Mismatch in sex between the external genitalia and gonads (testes/ovaries) named based on the genotype present Female pseudohermaphrodite (XX) due to excessive and inappropriate exposure to androgenic steroids during early gestation most commonly seen in congenital adrenal hyperplasia exogenous administration of androgens during pregnancy Male pseudohermaphrodite (XY) due to decreased effective testosterone concentration most commonly due to androgen insensitivity syndrome aka testicular feminization Presentation Physical exam female pseudohermaphrodite (XX) ovaries present external genitalia is virilized or ambiguous male pseudohermaphrodite (XY) testes present external genitalia is female or ambiguous
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