↑ Mineralocorticoids↓ Cortisol↓ Sex hormones↑ BP↓ K⁺Labs: ↓ androstenedione

Presentation: - XY: ambiguous genitalia, undescended testes- XX: lacks 2° sexual development

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).