Hairy elbows

Overview

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.

Symptoms

Hairy elbows

Causes

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)

Diagnosis

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?

Treatment

* 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)