Dubin-Johnson Syndrome Clinical Presentation

Updated: Dec 15, 2022
  • Author: Simon S Rabinowitz, MD, PhD, FAAP; Chief Editor: BS Anand, MD  more...
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History and Physical Examination


Patients with Dubin-Johnson syndrome (DJS) tend to develop nonpruritic jaundice during their teenaged years.

Although most patients are asymptomatic, some patients complain of nonspecific right upper quadrant pain, which has been attributed to the anxiety associated with prolonged diagnostic testing.

Subclinical cases can become evident following the initiation of oral contraceptives (which are known to impair organic anion transport), administration of estrogens as a component of gender reassignment, or during pregnancy.

Associated findings include the presence of hepatitis B virus (HBV)-related chronic hepatitis, a history of tubercular lymphadenitis, chronic cholecystitis, and coronary heart disease. A thorough family history can reveal a history of jaundice in an autosomal recessive pattern.

Reports have documented that patients with both Dubin-Johnson syndrome and hemolytic disease (eg, hereditary spherocytosis, [37] thalassemia [38] ) may experience worse jaundice than anticipated.

Physical examination

Nonpruritic jaundice is the most striking clinical feature of Dubin-Johnson syndrome. Aside from that, physical examination findings are generally normal, with the exception of possible hepatosplenomegaly.