Table 1.

Classification of AIS Phenotypes

TypeExternal Genitalia (Synonyms)Findings
CAISFemale (“testicular feminization”)
  • Absent or rudimentary wolffian duct derivatives
  • Absence or presence of epididymides and/or vas deferens
  • Inguinal, labial, or abdominal testes
  • Short blind-ending vagina
  • Scant or absent pubic and/or axillary hair
PAISPredominantly female (“incomplete AIS”)
  • Inguinal or labial testes
  • Clitoromegaly and labial fusion
  • Distinct urethral and vaginal openings or a urogenital sinus
  • Microphallus (<1 cm) with clitoris-like underdeveloped glans; labia majora-like bifid scrotum
  • Descended or undescended testes
  • Perineoscrotal hypospadias or urogenital sinus
  • Gynecomastia (development of breasts) in puberty
Predominantly male
  • Simple (glandular or penile) or severe (perineal) “isolated” hypospadias with a normal-sized penis and descended testes or severe hypospadias with micropenis, bifid scrotum, and either descended or undescended testes
  • Gynecomastia in puberty
MAISMale (“undervirilized male syndrome”)
  • Impaired spermatogenesis and/or impaired pubertal virilization
  • Gynecomastia in puberty

From: Androgen Insensitivity Syndrome

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Pagon RA, Adam MP, Ardinger HH, et al., editors.
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