A case of Dubin-Johnson syndrome with a pigmented neurofibroma

Clinically, Dubin-Johnson syndrome is characterized by mild icterus without specific symptoms or signs. The icterus is so mild that it is usually noted only during another illness, pregnancy, or the use of oral contraceptives. There is no pruritus in Dubin-Johnson syndrome. The physical examination is usually normal, except for the icterus, although hepatosplenomegaly is seen occasionally. Histologically, the liver is normal, except for the presence of dense pigment making it appear black grossly. Pigmentation of tissues other than the liver in patients with Dubin-Johnson syndrome has been reported only in a few cases. We experienced a case of Dubin-Johnson syndrome with extrahepatic pigmentation in the skin with a neurofibroma in a 66-year-old man.