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Definition

A form of male pseudohermaphroditism in which 46,XY males show ambiguous genitalia at birth, including perineal hypospadias and a blind perineal pouch, and develop masculinization at puberty. The name of the disorder stems from the finding of a blind-ending perineal opening resembling a vagina and a severely hypospadiac penis with the urethra opening onto the perineum.

Acronym

PPSH

Synonyms

5-ARD deficiency
Familial incomplete male pseudohermaphroditism type 2
Male pseudohermaphroditism due to 5-alpha-reductase deficiency

Keywords

Pseudohermaphroditism