46 Xx Disorder Of Sex Development Induced By Androgens Excess C5680227
A condition where an XX individual has excess male hormones causing atypical genital development. It affects both newborns and older individuals. Treatment may involve hormone management and surgical options.
Key facts at a glance
- ICD-10
- N50.9
- ICD-11
- GA00.0
- Body system
- reproductive
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Ambiguous genitalia at birth
External genitalia that appears neither typically male nor typically female, with partially fused labial-scrotal folds and enlarged phallic structure.
Immediately at birth for urgent specialist evaluation and appropriate gender assignment counseling.
Clitoromegaly
(reproductive)Abnormally enlarged clitoris due to prenatal androgen exposure, varying in severity from mild to significant enlargement.
Frequency: 85-95% of patients
Labial fusion
(reproductive)Partial or complete fusion of the labia minora or majora, creating a scrotal-like appearance.
Frequency: 70-80% of patients
Hirsutism
(dermatologic)Excess growth of coarse hair in male-pattern distribution, including face, chest, and back.
Frequency: 60-75% of patients
Severe neonatal acne
(dermatologic)Inflammatory acne presenting shortly after birth due to transplacental androgen exposure.
Frequency: 50-70% of patients
Accelerated growth velocity
(endocrine)Faster than normal growth rate in childhood due to early bone maturation from androgen exposure.
Frequency: 55-70% of patients
Premature pubarche
(endocrine)Early development of pubic hair before age 8, indicating early adrenarche from androgen excess.
Frequency: 50-65% of patients
Deepening of voice
(respiratory)Lower-pitched voice developing in childhood due to vocal cord thickening from androgen effects.
Frequency: 25-35% of patients
Primary amenorrhea
(reproductive)Absence of menstruation by age 15 or 3 years after secondary sexual characteristics develop.
Frequency: Variable, depends on virilization severity
Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Anti-Androgen Therapy
Medications such as spironolactone or other anti-androgen agents to block excess androgen effects. Used to manage hirsutism, acne, and other androgen-mediated symptoms.
Medication management with follow-up
Active treatment with regular monitoring by a healthcare provider is recommended.
Oral Contraceptive Pills
Combined hormonal contraceptives to suppress ovarian androgen production and regulate menstrual cycles. Provides predictable bleeding and reduces androgen effects.
Additional treatment options
Cosmetic Hair Management
Long-term hair removal strategies including laser therapy, electrolysis, or temporary methods. Addresses psychosocial impact of hirsutism and improves quality of life.
lifestyleBody Image and Self-Esteem Support
Therapeutic support addressing body image concerns, anxiety, and depression associated with visible androgen effects. May include cognitive behavioral approaches.
psychologicalCosmetic Procedures
Dermatological procedures for acne scarring, skin texture improvement, or other cosmetic concerns related to androgen excess effects.
procedureWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is 46 Xx Disorder Of Sex Development Induced By Androgens Excess C5680227?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Speiser PW, Arlt W, Auchus RJ, et al. Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: An Endocrine Society clinical practice guideline. Journal of Clinical Endocrinology and Metabolism. 2018.
Lee PA, Houk CP, Ahmed SF, Hughes IA. Consensus statement on management of intersex disorders. Pediatrics. 2006.
Hughes IA, Houk C, Ahmed SF, Lee PA. Consensus statement on management of intersex disorders. Archives of Disease in Childhood. 2006.
Witchel SF, Azziz R. Congenital adrenal hyperplasia. Journal of Pediatric and Adolescent Gynecology. 2011.
American Academy of Pediatrics. Technical report: Disorders of sex development. Pediatrics. 2008.
Miller WL, Merke DP. Congenital adrenal hyperplasia due to 21-hydroxylase deficiency. New England Journal of Medicine. 2020.
Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ. Williams Textbook of Endocrinology. 14th ed. Elsevier. 2020.
Mieszczak J, Houk CP, Lee PA. Disorders of sex development: Current perspectives. Current Opinion in Endocrinology, Diabetes and Obesity. 2009.
WHO. ICD-11 for Mortality and Morbidity Statistics: 6D50. Congenital adrenal hyperplasia. World Health Organization. 2022.
Khatchadourian K, Migeon CJ, Brown GA. Congenital adrenal hyperplasia. Expert Review of Endocrinology & Metabolism. 2011.
Medication Comparisons
Compare medications used to treat 46 Xx Disorder Of Sex Development Induced By Androgens Excess C5680227. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 27, 2026. · Read our editorial policy