↓ Mineralcorticoids↓ Cortisol↑ Sex hormones↓ BP↑ K⁺

Labs: ↑ renin activity, ↑ 17-hydroxyprogesterone

Presentation: - Most common- Presents in infancy (salt wasting) or in childhood (precocious puberty)- XX: virilization

All congenital adrenal enzyme deficiencies are characterized by skin hyperpigmentation (due to ↑ MSH production, which is coproduced and secreted with ACTH) and bilateral adrenal gland enlargement (due to ↑ ACTH stimulation).