Mzabi-Regaya Sabah, Chadli-Debbiche Aschraf, Ben Brahim Ehsen, Gritli Sami, Goutallier-Ben Fadhel Carole, Khalfallah Mohamed Tahar
Service d'Anatomie pathologique, Hôpital Mongi Slim, Tunisie.
Tunis Med. 2002 Apr;80(4):228-32.
The Dubin-Johnson syndrome (DJS) is an autosomal recessive liver disorder characterized by a chronic conjugated hyperbilirubinemia a dark greenish appearance of liver tissue, a double peaked sulfobromophthalein clearance curve, and a characteristic lysosomal accumulation of black pigment "melanine-like" in the hepatocytes. Laboratory datas indicated an increased urinary excretion of coproporphrin isomer I and leukotriene metabolites. In an effort to understand the morphological pattern and the pathogenesis of this disease we reviewed four cases of DJS.
