Dubin-Johnson syndrome is an inherited, relapsing, benign disorder of bilirubin metabolism.

Dubin-
Johnson syndrome is characterized by defective bilirubin excretion into bile. This results in reduced hepatic
bilirubin clearance. [1] This rare autosomal recessive condition is characterized by conjugated
hyperbilirubinemia with normal liver transaminases, a unique pattern of urinary excretion of heme
metabolites (coproporphyrins), and the deposition of a pigment that gives the liver a characteristic black
color (see the image below).

Gross liver specimen from a patient with

Dubin-Johnson syndrome showing multiple areas of dark pigmentation. Image courtesy of
Cirilo Sotelo-Avila, MD.