Varicella, usually a benign infection of childhood, is known to be associated with more serious complications, especially in adults and immunocompromised patients. Of these, varicella pneumonitis is the most common. Primary varicella hepatitis has been described, though rarely, in immunocompromised patients. We present a case of a previously healthy human immunodeficiency virus-negative 26-year-old immigrant from El Salvador in whom primary varicella developed that rapidly progressed to fulminant hepatic failure, disseminated intravascular coagulation, and death. Autopsy revealed evidence of varicella in the liver, adrenal glands, and myometrium. To our knowledge, this is the first report of a fatal case of varicella hepatitis in a previously healthy, immunocompetent patient.

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