Fatal visceral disseminated varicella zoster virus infection during initial remission induction therapy in a patient with lupus nephritis: a case report and review of the literature

Visceral disseminated varicella zoster virus (VZV) infection is a severe complication, characterized by a notably high mortality rate. Herein, we present a case of a 36-year-old-man involving visceral disseminated VZV infection that emerged during remission induction therapy involving high-dose prednisolone (PSL), mycophenolate mofetil (MMF), and hydroxychloroquine for lupus nephritis. Two months after starting the immunosuppressive therapy, he experienced a rapid onset of severe upper abdominal pain. The following day, clinical manifestations and laboratory abnormalities rapidly deteriorated. Hyperferritinemia and hypertriglyceridemia, indicative of hemophagocytic lymphohistiocytosis (HLH), emerged, along with escalating liver and renal impairment and newly appeared disseminated intravascular coagulation, and multiple organ failure is suggested. The patient developed widespread blistering predominantly on the trunk and face, patient's condition failed to ameliorate, ultimately culminating in his demise a few hours later. At autopsy, the cutaneous lesions with blisters revealed positive immunostaining with anti-VZV antibody, and similar findings were detected in multiple organs. HLH was confirmed in lymph nodes. It is crucial to emphasize the awareness of visceral disseminated VZV, particularly in cases patients are undergoing concurrent PSL therapy alongside MMF for SLE. The progression of this fatal condition usually begins with abdominal pain, followed by a skin rash a few days later. The present case is the first to show evidence of HLH occurring as a result of visceral disseminated VZV infection. This disease is extremely rare but extremely serious, therefore, VZV-DNA should be measured in cases where you suspect this disease for early diagnosis and treatment.