A Rare Cause of Portal Hypertension: Behcet’s Disease and Nodular Regenerative Hyperplasia of the Liver

Abstract

Nodular regenerative hyperplasia (NRH) is a rare liver condition in which widespread benign transformation of the hepatic parenchyma into small regenerative nodules occur, leading to development of non-cirrhotic portal hypertension. Conditions associated with NRH include rheumatologic, hematological, autoimmune, infectious, neoplastic, or drug-related etiology. Accurate diagnosis is made on liver biopsy, showing diffuse micronodular transformation in the absence of fibrosis. Here, we present the second case in world literature of a middle-aged man presenting with recent onset sleep reversal and memory loss with darkening of skin for four years associated with systemic manifestations, in whom porto-systemic shunt syndrome due to NRH secondary to Behcet’s disease was eventually diagnosed.