A 30-year-old man presents with unexplained fever, cervical lymphadenopathy, cutaneous rash on the anterior trunk, and erythematous palms of the hands. Laboratory findings include total bilirubin 6.9, AST 49, ALT 87, ALP 283, negative autoimmune serology, negative viral hepatitis serology, and a negative infectious disease workup. A liver biopsy was performed with the histologic features seen in the images. The iron stain was negative for hemosiderosis. The trichrome stain showed no significant fibrosis. The CMV and HSV immunostains were negative.
The correct answer is ...
Kawasaki disease (KD), previously known as mucocutaneous lymph node syndrome, is an acute, febrile, systemic vasculitis that usually effects infants and young children <5-years-old; however, some cases can occur in adults. The classic clinical presentation includes fever, mucocutaneous changes, and cervical lymphadenopathy. KD often involves the larger coronary arteries and is the most common cause of acquired heart disease in children. KD can also present with liver dysfunction and elevated transaminases, which is more common in adult-onset KD.
Histologically, the liver can show a vasculitis-induced chronic biliary tract disease pattern of injury. Medium-sized arteries and veins in the liver show injury secondary to inflammation. This can lead to ischemic-type injury to the biliary tree. Histologic findings include portal inflammation with increased neutrophils, acute cholangitis, bile ductular reaction, portal vein blood flow impairment, and Kupffer cell hyperplasia. Hepatic arterial flow insufficiency can also cause subtle hepatocellular change specifically manifested as concurrent increase in mitosis and apoptosis.
KD can commonly cause biliary tract injury with histologic findings that can appear similar to that of PSC, including portal inflammation, acute cholangitis, and bile ductular proliferation. However, classic PSC shows inflammation involving large ducts, causing chronic obstructive changes in the small portal tracts. Chronic cholestatic change and increased fibrosis, particularly peri-ductal concentric fibrosis, are characteristic findings. KD-induced bile duct injury is often acute linking to ischemia and infection. KD is the most correct answer.
The identification of portal granulomas brings sarcoidosis into the differential diagnosis. The clinical presentation and additional histologic findings makes sarcoidosis less likely. KD is the most correct answer.
KD can commonly cause biliary tract injury with histologic findings that can appear similar to that of PBC, including portal inflammation, granulomatous inflammation, and bile ductular proliferation. However, the characteristic histologic feature of PBC is florid ductal lesion with increased plasma-cell rich portal inflammation, with or without interface activity. To find features of chronic cholestatic change is also important for diagnosis of PBC. KD-induced bile duct injury is often acute linking to ischemia and infection. Given the negative autoimmune serology and clinical presentation of this patient, KD is the most correct answer.
Rachel Horton, D.O.
Fellow, Gastroenterology & Liver Pathology
Eric Chen, M.D., Ph.D.
Consultant, Anatomic Pathology
Associate Professor of Laboratory Medicine and Pathology
Mayo Clinic College of Medicine and Science