Case Series of Vaccine Associated Varicella Zoster Virus Infection in Immune Compromised Patients

Case series of vaccine associated varicella zoster virus infection in immune compromised patients

Vaccination with attenuated varicella zoster virus (VZV) can lead to infectious complications in immunodeficient patients. Here we report two immunocompromised patients who developed initial or reactivated vaccine strain varicella, leading to unique vesicular rashes involving the vaccinated limbs, without becoming wide-spread or remaining limited to a single dermatome.

Case 1- A 5 year old female presented with fever, jaundice, epistaxis and petechiae which began 2 weeks after receiving a second Varivax vaccination.  Diagnostic testing revealed pancytopenia and hepatitis with a peak ALT of 1712 unit/L. A liver biopsy showed diffuse portal and lobular hepatocellular inflammation with prominent lymphohistiocytic infiltrate.  A bone marrow aspirate and biopsy revealed hypocellularity and hemophagocytosis. She soon developed vesicles on her right arm and shoulder in a non-dermatomal pattern (Figure 1). The lesions remained localized to the entire right upper extremity, which was the site of the earlier Varivax vaccination. A vesicle was unroofed and the fluid tested positive for VZV by PCR.  Further analysis revealed the virus to be vaccine strain varicella.  She was treated with intravenous acyclovir and the lesions crusted over 21 days from presentation.

Case 2- A 17 month old female with Hurler Syndrome, on day + 6 after receiving an unrelated 7/8 Human Leukocyte Antigen matched cord blood transplant, developed a vesicular rash on her right thigh ,which was the site for Varivax vaccination 4 months prior to transplant.  A lesion was unroofed and the fluid revealed VZV by PCR. Subsequent analysis revealed the virus to be vaccine strain varicella. She was started on treatment with intravenous acyclovir and intravenous immunoglobulin and her lesions crusted over within 2 weeks.

These two cases highlight unusual presentations of vaccine strain varicella in immunocompromised patients. Vaccine strain varicella can cause a vesicular rash in a non-classical distribution. It is also notable that vaccine strain virus can trigger hemophagocytic lymphohistiocytosis in immune deficient patients. 

Figure 1. Entire vaccinated limb involved in a non dermatomal pattern