A rare autosomal recessive disorder in which individuals cannot synthesize endogenous ESTROGENS. In utero, this results in VIRILIZATION of both the fetus and mother, since the PLACENTA converts the precursor, DEHYDROEPIANDROSTERONE SULFATE, to TESTOSTERONE. Affected mothers exhibit HISUTISM and ACNE, which resolve following delivery. Affected female infants exhibit PSEUDOHERMAPHRODITISM at birth followed by cystic ovaries, delays in sexual development, and hirsutism at adolescence. Males may be asymptomatic at birth but can later exhibit tall stature, bone growth abnormalities and OBESITY, infertility and gynecomastia. Mutations in the CYP19A1 gene have been identified. OMIM: 613546
