PGIMER doctors report a rare case of Disseminated Varicella Infection

Dr Ashok K. Pannu and Dr Gopal Manikandan at Post Graduate Institute of Medical Education and Research, Chandigarh, India have reported a rare case of Disseminated Varicella Infection. The case has appeared in the New England Journal of Medicine.

Varicella-zoster is the virus that causes varicella (chickenpox), herpes zoster (shingles), and rarely, severe disseminated disease including diffuse rash, encephalitis, hepatitis, and pneumonitis. Disseminated disease is most often seen in immunocompromised patients. Varicella and chickenpox are common in children and are usually self-limited. However, immunocompromised hosts, especially patients with acute leukaemia receiving steroids, chemotherapy, or bone marrow transplantation, are at risk for severe varicella disease with dissemination and pneumonia or neurologic complications.

In the present case a 29-year-old man with a history of aplastic anemia who was being treated with cyclosporine presented to the emergency department with a 4-day history of fever, cough, and difficulty breathing at rest. One day before the onset of symptoms, a pruritic rash had developed on his face, trunk, and limbs. On physical examination, the pulse was 104 beats per minute, the blood pressure was 110/70 mm Hg, the respiratory rate was 36 breaths per minute, and the oxygen saturation was 90% while the patient was breathing high-flow oxygen. He had a diffuse rash at different stages of development, including papules, vesicles, pustules, and crusted vesicles (Panel A). Laboratory evaluation showed a platelet count of 33,000 per cubic millimeter (reference range, 150,000 to 400,000), an alanine aminotransferase level of 297 U per liter (reference range, 2 to 41), and an aspartate aminotransferase level of 281 U per liter (reference range, 2 to 40). A chest radiograph showed multiple nodules coalescing to form nodular consolidation and infiltrates in both lungs (Panel B).

Disseminated Varicella Infection

On the basis of his history of contact with a person who had chickenpox, along with the appearance of lesions and pulmonary symptoms in the context of immunosuppression, a clinical diagnosis of disseminated varicella infection was made. Despite treatment, which included intravenous acyclovir and mechanical ventilation with an acute respiratory distress syndrome protocol, the patient died 2 days after presentation.