Are there diagnostic histologic features of porphyria cutanea tarda in liver biopsy specimens?
In a blind review of 485 consecutive liver biopsy specimens, 16 from patients with porphyria cutanea tarda (PCT) and 469 from patients with other diseases, we have evaluated the frequency of the following features: a) needle-shaped cytoplasmic inclusions in liver cells, b) lobular aggregates of histiocytes laden with ceroid and iron, and c) the simultaneous presence of portal inflammation, hemosiderosis and fatty change, in order to ascertain their usefulness in the diagnosis of PCT. Cytoplasmic inclusions and lobular aggregates of macrophages were only found in two specimens, both of them from patients with PCT. The simultaneous presence of portal inflammation, hemosiderin and fat was found in nine patients (56%) with PCT and in nine (1.9%) of those without PCT. The localization of hemosiderin deposits differed between the two groups, being observed in periportal hepatocytes in cases with PCT, whilst it was predominantly found in Kupffer cells with a diffuse lobular distribution in cases without PCT. Thus, the finding in a liver biopsy specimen of hemosiderin in periportal hepatocytes, fatty changes and portal inflammation should alert one to the possible diagnosis of PCT and encourage the utilization of tests to confirm this diagnosis.
Campo
E
E
Liver Unit Hospital, Clinic i Provincial, University of Barcelona, Spain.
Bruguera
M
M
Rodés
J
J
D000328:Adult; D000368:Aged; D001706:Biopsy; D005260:Female; D006485:Hemosiderin / Q000032:analysis; D006644:Histiocytes / Q000473:pathology; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure; D007728:Kupffer Cells / Q000473:pathology; D008099:Liver / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D011164:Porphyrias / Q000175:diagnosis*; D012871:Skin Diseases / Q000175:diagnosis*
D006485:Hemosiderin