Sex: F
Vaccine: VARCEL
Symptoms: Herpes simplex test negative, Neck pain, Neutropenia, Neutrophil count decreased, Oedema
Clinical narrative: Information has been received from the authors of the article, concerning a 53-year-old woman who, on an unknown date, presented to the emergency department (ED) with a diffuse pruritic rash 17 days after receiving the varicella virus vaccine live (oka/merck) (manufacturer unknown) for prophylaxis (dose, dose number, and route of administration were not reported). The rash began on her chest and spread to her face, abdomen, back, and arms within 1 day. Her serologic test results before vaccination were negative for varicella-zoster virus (VZV), and she had no history of immunodeficiency or recurrent infections. She also had no other known exposure to VZV before the rash developed. She reported that she recently started taking meloxicam. Physical examination revealed 1-mm to 2-mm skin-colored vesicles, many with central erosion, located centrally on an edematous pink base on her face, trunk, and extremities. Her palms, soles, and mucous membranes were not affected. The physical examination results were within normal limits. In presentation, the patient's white blood cell count was 1.9x10^9/L, her absolute neutrophil count was 0.79x10^9/L, and her platelet count was 68x10^9/L. Her laboratory results before vaccination demonstrated a normal white blood cell count and platelet count of 127x10^9/L. Although the patient had a history of non-alcoholic steatohepatitis with mildly elevated aminotransferase levels, at the time of presentation to the ED, she had an alanine aminotransferase level of 86 U/L, and an aspartate aminotransferase level of 94 U/L. The patient reported a headache and mild neck pain for approximately 1 to 2 weeks before presentation, and a lumbar puncture was performed in the ED that showed normal cerebrospinal fluid cell counts and normal protein and glucose levels. The cerebrospinal fluid polymerase chain reaction was negative for VZV. Chest radiograph results were normal. Serologic test results for HIV, Epstein-Barr virus, and hepatitis B and C were negative. Two punch biopsies of the affected area were performed for histopathologic analysis and tissue culture. Histopathologic analysis revealed stratum corneum with a basket weave pattern. The epidermis contained single and clustered necrotic keratinocytes, and the dermis was found to have a mixed perivascular lymphohistiocytic infiltrate. Multiple necrotic keratinocytes with groundglass nuclei were seen in a hair follicle. The VZV immunostain was positive within the necrotic epidermal cells and within the affected hair follicle and the herpes simplex virus type 1 and type 2 immunostains were negative. Tissue culture was also positive for VZV. The biopsy specimen was cultured in H&V cells containing CV1 African green monkey kidney cells and MRC-5 human fetal lung cells. The live attenuated virus used in preparation of varicella virus vaccine live is propagated in MRC-5 cells and thus should replicate sufficiently in viral culture for detection in case of infection. On the same day the patient was admitted to the ED, she was treated for a presumed disseminated VZV infection. She received 740 mg of intravenous acyclovir every 8 hours for 2 days, after which her rash improved and her white blood cell and platelet counts increased. She was discharged home and completed 5 more days of oral acyclovir, 800 mg 5 times daily, for a total 7-day course of therapy. On follow-up with her primary care physician 7 days after discharge, her neutropenia and thrombocytopenia had resolved and her rash was almost completely gone. Her aminotransferase levels remained elevated. Given the rare nature of disseminated VZV infections after VZV vaccination in immunocompetent adults, this patient was referred to the immunology clinic for follow-up, but she declined this evaluation. The authors considered the disseminated varicella zoster vaccine virus infection to be related to varicella virus vaccine live (Oka/Merck) (manufacturer unknown). Upon internal review, the disseminated varicella zoster vaccine virus infection was determined to be medically significant.