Hirsutism
This booklet is intended as a practical guide to patient care for registrars, medical officers, interns and medical students attached to the gynaecology units at the University of the Witwatersrand Academic Hospitals. Anyone can use it.
Testosterone-secreting ovarian tumors
Congenital adrenal hyperplasia (CAH)
Symptomatic management for 'idiopathic hirsutism'
Many cases of hirsutism (90%) are idiopathic and require no more than symptomatic treatment or reassurance. Many such ‘idiopathic’ cases may have subtle hormonal synthetic errors (as in mild polycystic ovarian syndrome) or slighty altered sensitivity to androgens.
Pathological causes include polycystic ovarian syndrome, Cushing’s syndrome, congenital adrenal hyperplasia, 5-alpha reductase deficiency and testosterone-secreting tumors. Tumors should not be missed in these patients(Testosterone > or = 7nmol/L).
Clinical assessment
- A full general history and examination
- Onset of hirsutism - recent and/or sudden(suggests tumour)
- Menstrual regularity, irregularity
- Use of any medications
- Obesity suggests polycystic ovarian syndrome (PCOS)
- Hypertension, striae, and/or muscle wasting suggest Cushing’s syndrome(v rare)
- Pelvic mass suggests an androgen-secreting tumor but they are more likely too small to be palpable
- Extent of hirsutism (Ferriman-Galwey score(11 or 13 areas of the body – used in research)
- Virilism – voice changes, clitoromegaly, frontal balding, muscle bulk, and hirsutism
Investigations
- LH and FSH: a ratio of 3:1 suggests PCOS
- Testosterone:
- level of 2-5 nmol/L suggests PCOS
- level >6 nmol/L suggests an androgen-secreting tumor
- DHEAS:
- Slightly raised suggests PCOS
- Markedly raised suggests Cushing’s syndrome
- Does not usually affect management
- 17-hydroxyprogesterone levels if congenital adrenal hyperplasia is suspected
- Pelvic ultrasound scan to look for ovarian cysts or masses, or polycystic ovaries
CONDITIONS CAUSING HIRSUTISM
TESTOSTERONE-SECRETING OVARIAN TUMORS
Diagnosis
- These are rare but serious: androblastomas, arrhenoblastomas, lipid cell tumors, thecomas and luteomas
- Onset of hirsutism is sudden and marked; virilism is frequent
- Serum testosterone level >7 nmol/L is virtually diagnostic
- Ultrasound scan might not identify the tumor
- CT or even MRI scan may be required, or ovarian vein catheterisation
Management
- Surgical removal
CUSHING’S SYNDROME/DISEASE
Diagnosis
- Cushing’s syndrome - an adrenal adenoma or other cortisol-producing tumor
- Cushing’s disease - a pituitary ACTH-producing adenoma
- Cushingoid features, hirsutism and hypertension are frequent
- Early morning cortisol (>620 nmol/L), short and long dexamethasone suppression tests are diagnostic
Management
- Refer to specialist physicians
CONGENITAL ADRENAL HYPERPLASIA (CAH) - rare
Diagnosis
- Atypical maturity onset variant presents with hirsutism(salt losing death at childbirth is another)
- Frequently primary or secondary amenorrhoea
- Mild virilism or ambiguous genitalia may be found
- 17-hydroxyprogesterone level >20 nmol/L may be found
- 17-hydroxyprogesterone may rise only in response to ACTH stimulation
Management
- Refer to physicians
- Low-dose oral dexamethasone is the mainstay of medical treatment
- These individuals often consider themselves male and may be deeply traumatized by the diagnosis
5-ALPHA REDUCTASE DEFICIENCY
Diagnosis
- Very rare condition
- Primary amenorrhoea, and hirsutism or virilism particularly at puberty
- Mullerian agenesis (no uterus or cervix)
- Testosterone level is >6 nmol/L, with an XY karyotype
Management
Management by consultant and specialist clinic if available
Gonadectomy is recommended
POLYCYSTIC OVARIAN SYNDROME
This is a common cause of hirsutism and is discussed above as a cause of amenorrhoea.
SYMPTOMATIC MANAGEMENT FOR ‘IDIOPATHIC’ HIRSUTISM
- Treatment with anti-androgens takes 6 months to produce an effect
- Spironolactone 50-100 mg daily is effective
- Spironolactone must be taken with a non-androgenic combined pill to prevent pregnancy and possible feminization of a female embryo
- Cyproterone acetate (CA) can given as Diane-35 (CA 2 mg + EE 35 µg) or as Androcur (CA 10 mg on days 5-14 and estradiol 30 µg on days 5-15)
- Use of Androcur is cheaper than Diane-35. Diane is expensive
- Cosmetic measures, such as bleaching, plucking, shaving, electrolysis and laser may be considered, but the hospital does not provide these.