The disorder is ascertained either because of inguinal hernia (prepubertal) or primary amenorrhoea (postpubertal). Axillary and pubic hair are absent or scanty, but some vulval hair is usually present. Scalp hair is normal, and facial hair is absent. The external genitalia are unambiguously female, and the clitoris is normal. The vagina is short and blind-ending and may be absent or rudimentary. All internal genitalia are absent except for undescended testes that contain normal Leydig cells and seminiferous tubules without spermatogenesis (Fig. 11.30).
