Author(s)

Vaibhav Pandey, Vivek Srivastava, Anand kumar, Mumtaz Ansari, S. K. Singh

Department of Endocrinology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India.
Department of General Surgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India.
Department of Paediatric Surgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India;.

Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of familial Cushing's syndrome. It is characterized by bilateral adrenocortical hyperplasia with small to normal-sized adrenal glands containing multiple small adrenal cortical pigmented nodules [1,2]. PPNAD may occur in an isolated form or as familial PPNAD. Familial cases of PPNAD are usually associated with Carney complex in which Cushing’s syndrome is the most common endocrine manifestation [3]. Familial cases of PPNAD without associated Carney complex are very rare. Only a few cases of familial isolated PPNAD have been reported in the literature, mostly in females [4]. Isolated familial PPNAD has got a better prognosis than familial PPNAD associated with Carney Complex. This observation has important consequences for clinical management, follow-up and genetic counselling of such patients. Familial cases of PPNAD are rare and mostly present in females with associated Carney complex. We herein report a case of familial Cushing’s syndrome in male siblings due to PPNAD without associated Carney complex.

PPNAD; Carney Complex; Familial Cushing’s Syndrome

Pandey, V. , Srivastava, V. , kumar, A. , Ansari, M. and Singh, S. (2013) Familial primary pigmented nodular adrenocortical disease without Carney complex (CNC): A case report and review of literature. Case Reports in Clinical Medicine, 2, 454-456. doi: 10.4236/crcm.2013.28119.