July 2019

a 57-year-old white male with liver cirrhosis

Contributors: 

Mohammed Saad MBBS

PGY-3 Resident Physician, Anatomic Pathology and Clinical Pathology

Indiana University School of Medicine

Professional Interests: Cytopathology, Gastrointestinal Pathology, Hematopathology and General Surgical pathology

Ashley S. Inman, MD

Assistant Professor of Clinical Pathology & Laboratory Medicine

Indiana University School of Medicine

Clinical history: The patient was a 57-year-old white male with a history of decompensated liver cirrhosis. He was admitted to the hospital for bilateral lower extremity edema with erythema and severe volume overload from acute kidney injury. Laboratory investigation showed elevated liver enzymes, bilirubin, prothrombin time, ammonia, lactate, erythrocyte porphyrin (2895mCg/dl; NL: 0-35), total plasma porphyrin (260.5 mCg/dL; NL :< 1), and protoporphyrin fraction (251.7 mCg/dL; NL:<1). Additional lab findings included normal ceruloplasmin and alpha-fetoprotein. He was previously diagnosed with iron deficiency anemia. Microbiology studies were negative. However, serologic testing was positive for anti-nuclear antibodies and smooth muscle antibodies. During his hospitalization he continued to deteriorate and developed respiratory failure. Eventually, he was transitioned to comfort care only and ultimately expired one week after admission.

Gross examination: The post mortem external examination revealed jaundice, bilateral lower extremity pitting edema, skin erosions of various ages, and scars with patches of hyperpigmentation on both shins. The hands and fingers bilaterally were hyperpigmented and the dorsal skin was thickened and wrinkled (Image). The liver weight was normal, and the capsular surface showed diffuse discoloration with dark brown/maroon speckling. No focal lesions were identified with sectioning. The intrahepatic ducts and vessels were unremarkable. The common bile duct was not dilated and contained no stones or lesions. The gall bladder serosa was smooth and glistening. It contained 20 ml of viscous dark green bile and multiple dark green choleliths.

Image: left hand dorsum. Note thickened and wrinkled skin.

Microscopic examination and immunohistochemistry:

Sections from the liver show severe canalicular and intracellular cholestasis with deposition of an abnormal brownish-yellow pigment within the small and large bile ducts. The portal tracts are expanded by proliferating ductules associated with a neutrophilic infiltrate. There is bridging fibrosis. Within the lobules, scattered small foci of necrosis containing neutrophils are present as well as severe hepatocellular ballooning (Figure A-1, A-2, and A-3).

For PC users, right click to open image in a new window, then zoom to enlarge.

Figures A1 through A3: liver H&E section showing abnormal brownish yellow pigment within the small and large bile ducts

Fig. A1

Fig. A1 

Fig. A2

Fig. A2

Fig. A3

Fig. A3

Examination under polarized light demonstrates birefringent crystals, some of which are in a Maltese cross configuration (Figure B-1 and B-2). Histologic examination of skin from the dorsum of the hand shows thickening and hyalinization of the superficial dermal blood vessels that are highlighted with a PAS stain.

Fig. B1&B2: Examination of liver H&E section under polarized light showing birefringent crystals.

Fig.B1

Fig. B1

Fig. B2

Fig. B2

July 2019 Final Diagnosis and Discussion