Endoplasmic storage disease of liver: characterization of intracytoplasmic hyaline inclusions.

Two cases of endoplasmic storage disease of liver are described. The liver tissue in each case showed numerous intracytoplasmic hyaline inclusions of varying sizes with formation of ground-glass hepatocytes. These inclusions were pale eosinophilic in hematoxylin & eosin stained sections, and were periodic acid-Schiff and HBsAg negative. Immunoperoxidase studies revealed strong positivity for fibrinogen and complement components C3 and C4 in case 1 and exclusive positivity for fibrinogen in case 2. On electron microscopy, the inclusions appeared as granular or fibrillar material within dilated cisternae of rough endoplasmic reticulum.