SUPRARENAL HIRSUTISM
Testosterone, DHEA-S and androstenedione levels are increased. In this type of hirsutism, the patient presents hair growth in central areas of the body, with virilization, marked alopecia and low body weight.
The cause can be tumoral or non-tumoral.
NON-TUMORAL:
Congenital suprarenal hyperplasia: there is a high production of androgens in the suprarenal cortex.
An excess of androgens leads to the fusion of the labioscrotal folds, an increase in the size of the clitoris and anatomical changes of the urethra and vagina. The degree of this change depends on the exposure time, quantity and starting time of androgen action. The uterus, Fallopian tubes, and upper third of the vagina are normal. Differentiation of internal genitals is not affected because these structures are completely formed by the 10th week of gestation and the function of the suprarenal cortex is not significant until week 10-12. However, external genitals are not completely developed until week 20 and the increase of androgen can cause their masculinization. Deficit of 21-hydroxilase is one of the most frequent causes (95%) and also one of the most common congenital alterations. There is a deficit of cortisol and mineralocorticoids and an excess of precursors (pregnenolone, 17 OH progesterone, DHEA, androstenedione), which results in intrauterine virilization of the female foetus (female pseudohermaphroditism) and early pseudopuberty with growth acceleration and macrogenitosomia in males. An excess of androgens can manifest in the uterus, at birth or later in life.
CUSHING’S SYNDROME: in this syndrome androgen-dependent hirsutism is due to the production of ACTH by a tumour located inside or outside the hypophysis. Cushing’s syndrome is suspected when there is a combination of central obesity, high blood pressure, pinkish stretch-marks, intolerance to glucose, reddish, bloated face, osteoporosis and weak proximal muscles. In these cases hirsutism rarely comes in an isolated form. The best diagnostic criterion is elevated levels of free urine cortisol. If above 300 microg/24 hours it is nearly pathognomonic; if it is between 60 and 300 microg/24 hours a dexamethasone suppression test should be performed. Treatment will be based on the etiological diagnosis.
TUMORAL: DHEAS greater than 7,000 ng/dl.
SUPRARENAL ADENOMA
SUPRARENAL CARCINOMA: Suprarenal tumours are rare and more frequent in children and young adults, becoming extremely rare after menopause. These tumours rarely only secrete androgens; they usually produce cortisol and aldosterone as well. The alarming sign is the sudden development of intense generalized hirsutism. As regards diagnosis, it is basically based on elevated levels of SDHEA above 20 micromoles/l. Dynamic tests are not useful to detect these tumours as they act independently and do not respond to either the stimulation tests (ACTH) or to the suppression test (dexamethasone). Imaging techniques such as ultrasound and cat scan are very useful. Treatment for this condition is surgical.