Developmental Androgen Resistance Disorders of androgen action cause several types of male pseudohermaphroditism. In such disorders, testosterone formation and Mullerian regression are normal, and impairment of male development is a result of resistance to androgen action in the target cells. 5a-reductase deficiency is an autosomal recessive disorder characterised by: • Severe perineoscrotal hypospadias. • A blind vaginal pouch of variable size, opening either into the urogenital sinus or the urethra. • Testes with normal epididymides, vasa deferentia, seminal vesicles, and ejaculatory ducts that terminate in the blind-ending vagina. • A female habitus without female breast development but with normal axillary and pubic hair. • Absence of female urogenital structures. • Normal male plasma testosterone. • Masculinisation to a variable degree at the time of puberty.