106: Abnormal adrenal dehydro-epiandrosterone (DHA) production: a frequent, curable cause of hirsutism in childhood and adolescence ?

Abstract

The case of a 13 years old pubere girl with severe hirsutism and irregular menses is presented. The hirsutism was noted since infancy; puberty had started at the age of 9. She had no sign of virilism. Important hirsutism was also present in the mother. Markedly elevated plasma DHA level was found (934 ng/100ml); plasma testosterone (T) level was 34 ng/100ml. Urinary corticosteroids and plasma cortisol, 17 OH progesterone, ACTH, LH and FSH levels were normal. Under synacthen administration (1mg/day/3days) plasma DHA rose to 1 556 ng/100ml and plasma T to 51,9 ng/100ml, with a normal response of plasma cortisol (8 to 26 ug/100ml) and urinary 17 hydroxycoticosteroids (5,56 to 70,9 mg/24 h). Fluoxymesterone (20 mg/day/4days) did not induce any significant change in plasma DHA : 976 ng/100ml. By contrast low dosage of dexamethasone (DXM) (0,50 mg/ day/ 3 days) induced a major decrease in plasma DHA : 117 ng/160ml; there was no further decrease under high dosage of DXM (8mg/day/3 days) : 126 ng/100 ml. Long term treatment (15 months) with DXM (0,25 mg/day) was followed by a complete disappearance of the hirsutism and a normalisation of menstruations with plasma DHA level of 100 ng/100ml and plasma T level of 10,4 ng/100ml. Following this observation we have studied 4 similar cases of DHA dependant hirsutism without evidence of ensymatic defect : 3 adolescents 16 to 17 years old in whom hirsutism started at puberty and a 8 years old girl with a hirsutism present since infancy.

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