Background: Dubin-Johnson syndrome (DJS) is a rare clinical entity. We describe a case of DJS complicated by systemic lupus erythematosus (SLE).
Methods: A case of congenital hyperbilirubinemia with SLE was evaluated systematically including review of history, physical examination for the stigmata of chronic liver disease, and other investigations.
Result: Liver biopsy revealed a black liver with preserved architecture suggestive of DJS.
Conclusions: SLE may develop in DJS. The relationship between DJS and SLE in this case is most likely a chance occurrence.