ADRENOCORTICAL HYPERFUNCTION WITH SUDDEN ONSET OF HIRSUTISM

Abstract

SUMMARY 1. The investigation of a young girl who suddenly developed hirsutism is described. 2. Mild hirsuties of the legs had been noticed for 2 years, but a sudden increase in rate of growth of hair and the appearance of hair on her abdomen and around her nipples had occurred 4 months before examination. 3. The onset of hirsutism coincided with the onset of difficulties in a love affair. 4. The patient's excretion of steroids was investigated both before treatment and during treatment with oral cortisone and/or prednisone, and intramuscular corticotrophin (ACTH). The first series of tests were carried out while the patient was living her normal life, the second in hospital. The patient had a pattern of steroid excretion characteristic of many cases of idiopathic hirsutism and similar to that found in congenital pseudohermaphrodites with adrenal hyperplasia, although quantitatively different from the latter type of case. The excretion of C19-11-deoxy-17-ketosteroids (17-KS) was moderately raised, that of C19-11-oxy-17-KS greatly, and that of C21 metabolites of cortisol slightly raised. The excretion of 11 β-hydroxyandrosterone was greatly raised and the ratio 11 β-hydroxyandrosterone/11 β-hydroxy- and 11-ketoaetiocholanolone greatly increased, so that it lay outside the normal range for women. No suppression of adrenocortical secretion was achieved with cortisone while the patient was living normally, but was achieved with prednisone when she was in hospital. Unlike cases of congenital adrenocortical hyperplasia, the patient showed a rapid and considerable increase in the excretion of cortisol and its normal C21 metabolites when ACTH was given. 5. A 24 hr urine sample from the patient's twin sister showed the same abnormality of the C19-11-oxy-17-KS in their 5α/5β ratio (above), but the excretion rates of all 17-KS were about one-half of those found in the patient. The twin suffered from mild acne vulgaris only. 6. The estimated content of cortisol and 11 β-hydroxyandrostenedione in a sample of adrenal venous blood agreed reasonably well with the ratio of these substances calculated from the urinary estimations of their metabolites by a treatment similar to that of Dorfman [1954b]. It is concluded that over a certain range of excretion rates, Dorfman's type of treatment is valuable in inferring adrenocortical secretion rates, although more work is needed to establish the errors of such methods. 7. The clinical and biochemical findings are interpreted as supporting the hypothesis that hirsutism in this patient was due to an increase in adrenocortical secretion rate caused by emotional tension, coupled with a slight inherited abnormality in the ratio cortisol/androgens in her adrenocortical secretion. It is suggested that idiopathic hirsutism may commonly arise by this or similar mechanisms, the abnormal cortisol/androgen ratio giving no clinical signs until some factor causes an increase in adrenocortical secretion rate.