Hairy palms and soles

Overview

A rare inherited condition characterized by patches of hair on the palms and soles. The patches of skin associated with the hair has hair follicles and is different to the normal skin on palms and soles.

Symptoms

* Hairy palms * Hairy soles

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

* PCOS 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)