Androgen insensitivity syndrome classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aravind Reddy Kothagadi M.B.B.S[2]

Overview

Androgen insensitivity syndrome (AIS) represents a spectrum of defects in androgen action and can be subdivided into three broad phenotypes such as CAIS. PAIS and MAIS.

Classification

• Based on the degree of genital masculinization, Androgen insensitivity syndrome is divided into three categories:

• When the external genitalia is that of a normal female it is classified as Complete androgen insensitivity syndrome (CAIS)
• When the external genitalia is that of a normal male it is classified as Mild androgen insensitivity syndrome (MAIS)
• When the external genitalia is partially, but not fully masculinized it is classified as Partial androgen insensitivity syndrome (PAIS)

• Individuals with both Partial androgen insensitivity syndrome (PAIS) and Complete androgen insensitivity syndrome (CAIS) have 46 XY karyotypes.

Classification of Androgen Insensitivity Syndrome Phenotypes
Type	External Genitalia	Findings
CAIS - (Complete androgen insensitivity syndrome)	Female (“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.
Predominantly female (“incomplete AIS”)	Inguinal OR labial testes. Clitoromegaly and labial fusion. Distinct urethral and vaginal openings OR a urogenital sinus.
PAIS - (Partial androgen insensitivity syndrome)	Ambiguous	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.
MAIS - (Mild androgen insensitivity syndrome)	Male (“undervirilized male syndrome”)	Impaired spermatogenesis AND/OR impaired pubertal virilization. Gynecomastia in puberty.