Congenital adrenal hyperplasia and children

Congenital adrenal hyperplasia consists of a heterogenous group of inherited disorders due to enzymatic defects in the biosynthetic pathway of cortisol and/or aldosterone. This results in glucocorti-coid deficiency, mineralocorticoid deficiency and androgen excess. 95% of CAH cases are due to 21-hydroxylase deficiency. Clinical forms range from severe classical CAH associated with complete loss of enzyme function to milder non-classical forms (NCAH). Excess Androgen affects the pilosebaceous unit, causing cutaneous manifestations such as acne, androgenetic alopecia and hirsutism.