A patient with Carney’s complex (CAR47.01) with the germline IVS2+1 G>A PRKAR1A mutation. (a) Since childhood the patient had freckling on the vermillion border of the upper lip (lower arrow) and blue nevi on the face (upper arrow) and elsewhere. (b) Extensive genital pigmented nevi and lentigines (arrow). (c) Pigmentation of the inner canthus that is pathognomonic for Carney’s complex. (d) The patient first presented with a stroke (arrow) and right-sided paralysis due to dislodged right atrial cardiac myxoma. (e) She developed Cushing’s syndrome due to primary pigmented nodular adrenocortical disease, which is characterized by the many brown micronodules (arrows) present throughout the adrenal cortex. (See also Fig. 6.15.1)