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Hyper-IgE syndrome (HIES) is a primary immunodeficiency disorder characterized by eczema, cold abscesses, pneumonia, eosinophilia, and a very high serum IgE concentration. An association with celiac disease is rare. Immunodeficiency and autoimmunity are two manifestations of immune system dysfunction that can be associated with common pathophysiological links. We present the case of a 3-year-old child with psychomotor retardation and a history of recurrent infections who had generalized eczema, failure to thrive, and abdominal distension with hepatosplenomegaly. The patient improved after receiving a monthly intravenous immunoglobulin infusion and a gluten-free diet.

Autoimmune Hepatitis (AIH) pathogenesis is still unknown. However, among patients who have a genetic susceptibility, some viral infections appear to be triggers for AIH. We report a case of a child who developed type 2 autoimmune hepatitis as a result of HEPATITIS A Virus (HAV) infection. An 8-year-old female who was diagnosed with hepatitis A three years ago and was cured after three months. She developed jaundice and epistaxis a few years later. Complementary tests revealed polyclonal hypergammaglobulinemia, antinuclear and anti-LC1 antibodies, and hepatocyte necrotico-inflammatory lesions in a liver biopsy. The diagnosis of type 2 autoimmune hepatitis was kept. A remission was obtained under corticosteroid and azathioprine treatment. In cases of persistent HAV infection, AIH should be investigated so that therapy can start earlier. To prevent more serious complications, AIH patients should also receive the Hepatitis B Virus (HBV) and HAV vaccines.