Dubin-Johnson syndrome

Dubin-Johnson syndrome is an autosomal recessive condition characterized by jaundice. Total bilirubin levels usually range between 2 and 5 mg/dL, with increased plasma conjugated bilirubin.

A dark pigment accumulates in the liver, giving it a characteristic black color.

Primary defect is a mutation in an apical canalicular membrane protein responsible for excretion of bilirubin.

Jaundice is worsened by an intercurrent illness, pregnancy, and oral contraceptives.

A unique pattern of urinary excretion of coproporphyrins (by-products of heme synthesis) aids in the diagnosis.

It is a benign condition with a normal life expectancy and no specific treatment is required.

Dubin-Johnson syndrome (DJS) is an autosomal recessive condition manifested by chronic conjugated hyperbilirubinemia and accumulation of a dark pigment in the liver parenchymal cells. It is a benign condition with normal life expectancy.[1]Dubin IN, Johnson FB. Chronic idiopathic jaundice with unidentified pigment in the liver cells: a new clinicopathologic entity with report of 12 cases. Medicine. 1954 Sep;33(3):155-97. http://www.ncbi.nlm.nih.gov/pubmed/13193360?tool=bestpractice.com [2]Sprinz H, Nelson RS. Persistent nonhemolytic hyperbilirubinemia associated with lipochrome-like pigment in liver cells: report of 4 cases. Ann Intern Med. 1954 Nov;41(5):952-62. http://www.ncbi.nlm.nih.gov/pubmed/13208040?tool=bestpractice.com