It seems that hypercortisolism in PMAH is related to the increased number of adrenocortical cells supposedly caused by mutations in ARMC5 and the contribution of aberrant G-coupled receptors and intra-adrenal ACTH in hyperplastic adrenal cells is suggested by mechanisms of the autonomous cortisol production in PMAH [34,41,42]. This evidence concerns the gene POMC and adrenal gland hyperfunction.