We report on a 30-year-old Japanese woman with granulomatosis with polyangiitis (GPA) complicated by pituitary diabetes insipidus and multiple lung granulomas. The granulomas disappeared with prednisolone (50 mg/day) and rituximab, although continuous nasal desmopressin was needed to control diabetes insipidus after immunosuppressive therapies. At the time of presentation, the patient had abdominal pain and disseminated intravascular coagulation but no rash. She died of continuous hemorrhage from her skin of neck, mucosa of her pharynx, and small intestine. At autopsy, varicella zoster virus (VZV)-DNA detected in serum and VZV antigens detected in tissues of her pharynx, esophagus, and liver led to a diagnosis of visceral disseminated VZV infection (VD-VZV). She also complicated cytomegalovirus infection in her stomach and ovaries. Her posterior pituitary gland had been replaced by foamy macrophages.
In 38 reported cases of VD-VZV, rash appeared following the onset of abdominal pain (mean interval, 6.5 days) but was lacking in 11% of cases. The mortality rate associated with VD-VZV was as high as 29% and survived cases were treated with antivirals earlier than mortal cases. A quick diagnosis with detection of VZV-DNA or VZV antigens in sera or tissues using PCR or immunohistochemistry examination and early empirical treatment with antivirals are important.