A rare condition where the elbows become progressively hairy until about 5 years of age and then slowly disappears. Short stature appears to be associated in the some of the few cases reported.
o Drug-induced –Cyclosporin, steroids, oral contraceptives, Dilantin, some diuretics (acetazolamide, hydrochlorothiazide), Minoxidil, penicillamines * Syndrome-associated –Cornelia de Lange syndrome –Trisomy 18 –Hurler syndrome –Bloom syndrome –Seckel syndrome –Marshall-Smith syndrome –Rubinstein-Taybi syndrome –Leprechaunism * Ovarian –PCOS –Gonadal dysgenesis –Ovarian tumors * Adrenal –CAH –Cushing syndrome –17α-hydroxylase deficiency –21-hydroxylase deficiency –Adrenal tumor * Other causes –Idiopathic –5 α-reductase deficiency –Hyperprolactinemia –HAIR-AN syndrome (hirsutism, androgenization, insulin resistance, and acanthosis nigricans) –Achard-Thiers syndrome: Obesity and facial hirsutism develop by 15–30 years of age; hypertension and obesity occur later –Porphyria: Congenital erythropoietic porphyria have increased body hair, red urine, photosensitivity with bullae, and red to pink teeth (werewolves of old)
The routine diagnostic workup includes a serum free testosterone, free cortisol, prolactin, a skull x-ray (much more economical than a CT scan or MRI of the brain), and a urinary gonadotrophin assay. If a pituitary tumor or lesion is strongly suspected, an FSH and LH should be done regardless of results of routine tests. An overnight dexamethasone test is more accurate than a routine free cortisol in diagnosing Cushing's syndrome. Pelvic ultrasound and CT scan of the abdomen would complete the workup, but why order these expensive diagnostic tests before consulting a gynecologist or endocrinologist?
* COS and idiopathic –Estrogen-predominant oral contraceptives decrease plasma androgens –Depo-Provera may also be used –Spironolactone works by competing for androgen receptors –Cimetidine has a side effect of decreasing testosterone and increasing estrogen levels –GnRH agonist to facilitate better function of the hypothalamic-pituitary axis * Drug-induced: Eliminate causative medication if possible and hirsutism may resolve spontaneously * Hyperprolactinemia: Bromocriptine (prolactin antagonist)