Sex: M
Vaccine: VARCEL
Symptoms: Blister, Blood immunoglobulin G normal, Disseminated varicella zoster vaccine virus infection, Immunodeficiency, Injection site vesicles
Clinical narrative: Information has been received from authors of a published article. This literature marketed report was concerning a 15-month-old male infant. The patient had a history of development delay and weight loss with multiple hospital admissions was diagnosed with a pustular lesion identified as vaccine strain varicella at the vaccination site. He was ultimately determined to be HIV infected following a protracted evaluation, which did not initially include HIV since his mother was confirmed HIV seronegative at 3 months gestation. The patient's first inpatient admission was at 12 months of age for evaluation of developmental regression. He presented with loss of milestones including initially loss of the ability to stand, then the ability to sit unsupported, followed by loss of head control. He was reported to have normal development until the age of 9 months, with ability to lift his head and chest at 2 months, sit unsupported at 5 months, stand with assistance at 9 months, and say "mama" at 11 months. He had recurrent otitis media and respiratory infections between 9 and 11 months of age. During the initial admission, a head computed tomography (CT) scan demonstrated increased density within the subcortical white matter, pineal gland, and possibly basal ganglia. Magnetic resonance imaging of the brain showed nonspecific subtle, symmetric T2 hyperintensities of both globi pallidi. The second hospital admission at 14 months of age was for evaluation of fever to 38.8 celsius degree, cough and increased work of breathing. He was diagnosed with possible viral bronchiolitis and transiently required 1 L/min of supplemental oxygen. He was discharged the following day without an infectious etiology being determined. The third hospitalization was for fever and cough. He was initiated on meropenem for aspiration pneumonia and a repeat dysphagia study demonstrated worsening aspiration of thick liquids, which he had previously successfully consumed. A gastrostomy tube with fundoplication was performed. His antibiotic coverage was broadened to include vancomycin and fluconazole. Postoperatively, he developed acute respiratory distress syndrome with a maximum partial pressure of arterial oxygen/fraction of inspired oxygen ratio of 106 and oxygenation index of 24. He had persistent fevers, and his respiratory cultures grew Stenotrophomonas maltophilia and sulfamethoxazole (+) trimethoprim was added to his antibiotic regimen. Fluconazole was changed to micafungin due to worsening transaminitis. He developed systemic capillary leak with abdominal compartment syndrome necessitating a peritoneal drain, with negative peritoneal fluid cultures. He remained intubated for 20 days in total. On an unknown date (reported as "2 months before appearance of lesion"), the patient vaccinated with a dose of varicella virus vaccine live (manufacturer unknown) in the left thigh (route, dose, lot# and expiration were not reported) for prophylaxis (contraindicated product administered). Given the lack of a unifying diagnosis, the patient received whole genome sequencing, which failed to reveal a genetic etiology. Antibiotics were discontinued after 31 days of meropenem, 11 days of vancomycin, 5 days of fluconazole and 10 days of micafungin. The day after cessation of antibiotics, the patient developed a large bullous 2 X 2 cm lesion with yellow fluid on the lateral aspect of the left thigh. Subsequently, he developed small vesicular lesions in the left inguinal area and on the left posterior shoulder. A Tzanck smear was performed on the lesion, which was consistent with varicella zoster infection, which was later confirmed by polymerase chain reaction. He was initiated on acyclovir 60 mg/kg/d intravenously. The rash evolved into a macular lesion with rare surrounding vesicles. The combination of disseminated varicella zoster virus with a history of varicella zoster virus vaccine administration and no exposure to wild-type varicella zoster was considered highly suggestive of an immunologic defect in the cellular immune response. Varicella Zoster Quantitative polymerase chain reaction revealed 631 copies/ml from serum 2 days after the rash appeared. Genotyping was determined the virus was the vaccine strain. Workup was notable for decreased immunoglobulin G (IgG) (452 mg/dl) suggestive of a broad immunodeficiency. He was therefore evaluated for HIV type-1 infection despite his mother's negative test in the first trimester of pregnancy. The outcome of the event was not reported, the reporter considered the event to be related to varicella virus vaccine live (manufacturer unknown). Upon internal review, disseminated varicella zoster vaccine virus infection was determined to be medically significant.