INTRODUCTION: Varicella-zoster infection is typically a self-limited disease of childhood. Disseminated varicella-zoster occurs more commonly in immunocompromised patients and carries a mortality rate of 80%. We report a case of disseminated varicella-zoster that presented with severe right upper quadrant (RUQ) pain several days before the rash in a patient with chronic lymphocytic leukemia (CLL).
CASE PRESENTATION: A 59-year-old woman presented to another institution with five days of severe RUQ pain not related to food intake. She had history of cholecystectomy and CLL in remission (last chemotherapy six months before admission). Physical exam was pertinent for RUQ tenderness. The initial laboratory workup, including liver enzymes, was unrevealing. Imaging studies including CT abdomen, IV pyelogram, and Endoscopic Retrograde Cholangiopancreatography were normal. A biliary stent was placed despite patent bile ducts. Patient developed thrombocytopenia and a petechial rash over the trunk and extremities. On admission to our institution, small red papules were noticed in the trunk, buttocks and vaginal area. Biopsy showed purpura but no vasculitis. Patient developed elevated transaminases and direct bilirubin. Peripheral smear showed toxic changes in white cells. Autoimmune and viral markers were negative. She required mechanical ventilation for worsening mental status and hypoxemic respiratory failure. Skin lesions became crusty with concomitant active lesions in different areas. Given high suspicion for disseminated varicella-zoster, acyclovir was started. A second skin biopsy showed intranuclear viral inclusions consistent with varicella-zoster infection. Intravenous Immunoglobulin (IVIG) was started given severity of disease, based on evidence from isolated case reports. She completed two weeks of acyclovir and five days of IVIG with dramatic clinical improvement. She was started on high-dose prednisone as thrombocytopenia was felt secondary to an autoimmune process triggered by the viral infection. Her liver enzymes, platelet count, hypoxemia and mental status improved significantly. She was extubated and discharged a few days later.
DISCUSSION: Varicella-zoster infection is typically self-limited, presenting with fever and malaise for one to two days, and followed by a vesicular rash. Patients with profound immunosuppression are at the greatest risk of dissemination. Organs usually involved are the lungs, liver and central nervous system. The case we present was unusual, as it presented with severe RUQ pain and was complicated by pneumonia, hepatitis, thrombocytopenia and central nervous system involvement. There are some reports of diffuse abdominal pain as the presenting symptom for disseminated varicella-zoster in immunosuppressed patients.1 To the best of our knowledge, this is the first report of disseminated varicella presenting with RUQ pain. We believe the cause of the abdominal pain was neuropathic, as the workup including blood and imaging studies was unremarkable. The pulmonary involvement was likely varicella-zoster pneumonitis as chest radiography showed diffuse interstitial infiltrates and respiratory cultures were negative before antimicrobials were started. The liver abnormalities were secondary to varicella-zoster hepatitis as the initial liver enzymes, viral workup and imaging studies were unremarkable. Varicella-zoster is notorious for causing autoimmune hematologic abnormalities2, this patient developed thrombocytopenia that could not be otherwise explained and resolved with treatment. Although we could not document varicella-zoster in the CSF, we strongly believe she had varicella-zoster encephalitis given the disseminated presentation. The atypical appearance of the rash raised other possibilities like leukocytoclastic vasculitis and TTP that were ruled out by our extensive workup.
CONCLUSIONS: Varicella-zoster should be considered in the differential diagnosis of immunocompromised patients presenting with abdominal pain, especially when blood and imaging studies fail to reveal the etiology. The presented case is unique because of its presentation, severity of disease and response to therapy.
Reference #1 Magi E. Severe varicella in an immunocompromised adult presenting with abdominal pain. West J Med 2000;173:376-7.
Reference #2 Ali N. Chickenpox associated thrombocytopenia in adults. J Coll Physicians Surg Pak 2006;16:270-2.
DISCLOSURE: The following authors have nothing to disclose: Angel Coz Yataco, Ayan Sen, Rana Awdish
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