Fibrosis around the portal triads. Bile duct proliferation.

Onion-skin fibrosis around a bile duct. Very suggestive of PSC. It took me a long time to find a case of PSC with this textbook picture. Please take a moment and appreciate.

A closer look at this onion-skinning fibrosis. As the disease advances, the bile duct is obliterated and replaced by a scar.

PAS stain demonstrates slightly thickened basement membranes of the bile ducts in some areas, but this is probably not the best example.

Copper stain shows focal copper deposition adjacent to triads.

This patient developed cholangiocarcinoma. there is cirrhosis and a duct here shows concentric fibrosis Image

many ducts are obliterated and you can see where they used to reside as a pink amorphous area Image

ANother case, maybe can delete the other images that are less good Image

2 Image

cholestasis Image

inflammation Image

scar Image

destructive venulitis Image

This particular 50 year old male patient had a longstanding history of inflammatory bowel disease (ulcerative colitis) and had a cholestatic picture (increased alkaline phosphate and markedly increased GGT). These a strong clues for primary sclerosing cholangitis.

Histologically, a fibro-obliteration of the bile ducts is seen. The classic onion-skin fibrosis of the bile duct (if present) is extremely helpful. PAS staining will occasionally show thickened basement membranes of the bile duct.

Patients with PSC often have abnormalities in copper metabolism, which can be demonstrated by copper accumulation in hepatocytes.

→70% of patients with PSC have inflammatory bowel disease.

→Fibrosis around portal triads, especially "onion-skin" fibrosis, is the typical histologic picture.