complete androgen insensitivity syndrome
ICD-10: E34.1ORPHA: 99429
Overview
intersex variation where one has no response to androgens and is born with a vulva and clitoris
Available Treatments (0)
No treatments linked yet
Clinical trials or compassionate use may be available — consult a specialist.
Clinical Presentation
Signs and symptoms associated with complete androgen insensitivity syndrome, sourced from HPO and Orphanet clinical annotations.
Increased serum testosterone levelBlind vaginaAnxietyDelayed pubertySparse axillary hairAbsent axillary hairSparse pubic hairAbsent pubic hairIncreased circulating antimullerian hormone concentrationDepressionTesticular neoplasmAcneAbnormal circulating follicle-stimulating hormone concentrationGerm cell neoplasiaAbnormal morphology of female internal genitaliaTall statureAplasia of the uterusPrimary amenorrheaMale infertilityAplasia/Hypoplasia of the fallopian tubeBilateral cryptorchidismFemale external genitalia in individual with 46,XY karyotypeElevated circulating luteinizing hormone levelAbnormal uterine cervix morphologyIncreased serum estradiol
Classification & Codes
ICD-10 Code
E34.1Orphanet Code
ORPHA:99429complete androgen insensitivity syndrome
| ICD-10 | E34.1 |
| Orphanet | ORPHA:99429 |
| Treatments | 0 drug(s) |
| Symptoms on record | 25 signs |
| Status | published |
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO