Steroid responsive hypertension secondary to 11-beta hydroxylase deficiency--a case report.

BACKGROUND: Congenital adrenal hyperplasia (CAH), due to deficiency in 11â hydroxylase enzyme is rare a cause of virilization associated with glucocorticoid responsive hypertension. OBJECTIVE: To present a rare cause of CAH and secondary hypertension responsive to glucocorticoid therapy. METHODS: Clinical and laboratory evaluation of a young woman with a "phallus" and hypertension. Investigations carried out included serum biochemistry, bone age determination, sex chromatin evaluation and serum levels of androgens and 17-OH progesterone, as well as pelvic ultrasonography. RESULTS: There was a history of cliteromegaly, first noticed at age 14 years, but menstrual periods were said to be regular. Physical findings included male type hairline and musculature with a blood pressure of 160/110mmHg. Breast development was Tanner Grade 3 while the public hair was male in pattern. The clitoris was 5cm long. Serum electrolytes were normal but levels of testosterone, dehydroepiandosterone sulphate and 17-OH progesterone were elevated. Features were compatible with a diagnosis of congenital adrenal hyperplasia (CAH) due to Il-beta-hydroxylase defficiency. Hypertension responded to glucocorticoid therapy, recurred on default and was corrected again on re-starting hydrocortisone. CONCLUSION: Congenital adrenal hyperplasia due to 11â-hydroxylase deficiency though rare, should be considered in the differential diagnosis of hypertension with virilization in young females.