Question: A 35-year-old woman presented with a 1-week history of fevers, right upper quadrant and epigastric pain, and a non-pruritic, non-painful rash. Her past medical history was significant for a diagnosis of human immunodeficiency virus (HIV) 5 years prior, not currently on treatment, and hypertension, on Lisinopril. She had received highly active antiretroviral therapy (HAART) for one year after diagnosis but was subsequently lost to follow-up. Her last known CD4 count was 410 cells/mcL four years prior to presentation. Upon presentation, vital signs were within normal limits except for a fever of 38.8°C. Physical examination was notable for multiple crusted erosions and papules present on her face, abdomen, back, and extremities (Figures A–C). Laboratory evaluation demonstrated the following: alanine aminotransferase level 291 U/L, aspartate aminotransferase level 260 U/L, alkaline phosphatase level 135 U/L, total bilirubin 0.4 mg/dL, hemoglobin 12.9 g/dL, 2% blasts on peripheral smear, leukocytes 8 x 109/L, and platelet count 132 x 109/L. CD4 count was 118 cells/mcL and HIV-1 RNA 4,120,000 copies/mL. Chest x-ray demonstrated slight nodular peribronchovascular densities in both lungs and a right upper quadrant ultrasound demonstrated non-specific gallbladder wall edema. Computed tomography abdomen/pelvis was notable for diffuse heterogeneity of the hepatic parenchyma and innumerable small hypodensities in the liver and spleen, some with subtle peripheral enhancement. Which of the following is the most appropriate initial treatment for this patient’s condition?A.Supportive careB.Intramuscular penicillin G benzathineC.Intravenous acyclovirD.Highly active anti-retroviral therapy (HAART) Look on page 493 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image(s) to Practical Teaching Cases. The correct answer is C. Intravenous acyclovir for disseminated varicella zoster virus infection in an immunocompromised host The differential for fever and widespread rash in an immunocompromised individual is broad, and includes disseminated herpes simplex virus, varicella zoster virus (VZV), secondary syphilis, bacillary angiomatosis, disseminated gonococcal infection, parvovirus, acute hepatitis B, cryptococcosis, histoplasmosis, blastomycosis, and others. Our patient underwent liver biopsy and pathology demonstrated hepatic parenchyma with portal and lobular inflammation but no necrosis (Figures D, E). VZV polymerase chain reaction (PCR) from the liver returned positive. Moreover, swab of facial lesion was performed with positive VZV PCR. A diagnosis of disseminated varicella zoster infection was made based on the biopsy results along with multi-organ involvement including pulmonary and hepatic manifestations. Notably, her VZV IgM and IgG were negative, indicating the need for a high degree of suspicion if the clinical scenario is consistent with VZV. While uncomplicated VZV can be treated with supportive care or oral antivirals, such as acyclovir, valacyclovir, or famciclovir, an immunocompromised host with disseminated VZV should typically be admitted to the hospital for intravenous acyclovir.1Ahmed A.M. Brantley J.S. Madkan V. et al.Managing herpes zoster in immunocompromised patients.Herpes. 2007; 14: 32-36PubMed Google Scholar Answer B would be appropriate if secondary syphilis was suspected. In this case, initiation of HAART (answer D) before treatment of VZV could increase the risk of immune reconstitution inflammatory syndrome and exacerbate her pneumonitis and hepatitis. Complications of VZV infection include post herpetic neuralgia, herpes zoster ophthalmicus or oticus, acute retinal necrosis, meningitis, and encephalitis. In immunocompromised hosts, VZV can present with cutaneous dissemination and visceral organ involvement with pneumonia and hepatitis. Biopsy proven VZV hepatitis is rare and is associated with a high mortality.2Anderson D.R. Schwartz J. Hunter N.J. et al.Varicella hepatitis: a fatal case in a previously healthy, immunocompetent adult. Report of a case, autopsy, and review of the literature.Arch Intern Med. 1994; 154: 2101-2106Crossref PubMed Google Scholar,3Sherman R.A. Silva Jr., J. Gandour-Edwards R. Fatal varicella in an adult: case report and review of the gastrointestinal complications of chickenpox.Rev Infect Dis. 1991; 13: 424-427Crossref PubMed Scopus (22) Google Scholar Fortunately, our patient did not develop fulminant hepatic failure. She was treated with 4 days of IV acyclovir with improvement in her fevers and transaminitis and then transitioned to oral valacyclovir. HAART was also initiated two days after the start of IV acyclovir. At a follow-up visit one week later, she continued to improve and had no recurrence of symptoms. We would like to thank Dr Taofic Mounajjed for his contribution of pathology photos and interpretation to this case.
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Nov 26, 2019
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