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Aetiology

  • Genotype XY as should be in the male.
  • Testes develop normally and secrete Androgens and MIH
  • Foetal genetalia are genetically insensitive to the actions of Androgens due to a point mutation in the Androgen receptor.

Consequence

  • Wolffian Ducts regress, allowing formation of female external genetalia:
    • Labia
    • Clitoris
    • Vagina
  • Sertoli cells are present and continue to secrete MIH, causing Mullarian Duct regression.
  • Since both Wolffian AND Mullarian Ducts regress, there is no internal genetalia.