Disseminated Zoster in an Immunocompromised Patient
“Shingles (herpes zoster) is a secondary VZV infection, most common in the elderly and/or immunocompromised…”
CASE
A 72-year-old woman with a history of rheumatoid arthritis (RA), on methotrexate and Upadacitinib, presented to the emergency department with right sided, burning abdominal pain and associated painful, itching rash. She also reported lesions on her chest, back, and lower face, which had cropped up over the course of the last two to three days. She stated that she first developed pain that “felt like needles.” She subsequently developed the rash. Review of systems was otherwise unremarkable. She denied any new medications. She recalled either having measles or chickenpox as a child but was unsure which. She had not received the shingles vaccine.
Vital signs were within normal limits. On examination, she was not toxic appearing, but appeared uncomfortable. She had multiple scattered erythematous, vesicular lesions. Some lesions had active blisters, whereas others had overlying scabbing. The lesions involved multiple dermatomes, including the right lower jaw, the right and left chest wrapping around to the back linearly, and the right lower abdomen with associated lumbar rash. There was no mucus membrane involvement. The remainder of a complete physical exam was unremarkable. The patient was immediately placed on contact and airborne precautions. Laboratory evaluation, including a complete blood count, basic metabolic panel, hepatic function panel, lipase, urinalysis, and respiratory pathogen panel were unremarkable. A chest x-ray revealed no acute process. Blood cultures were drawn and sent. The lesions were swabbed for varicella zoster virus (VZV) and herpes simplex virus (HSV). The patient was started on intravenous acyclovir and lactated ringers fluids, given acetaminophen for pain, and admitted to the inpatient medicine service for further management.
DIAGNOSIS
Disseminated herpes zoster (shingles), also referred to as secondary VZV, was thought to be the most likely diagnosis in this patient, given her age and immunocompromised status. Primary VZV (chickenpox) was considered but thought to be less likely due to the patient’s age and lack of systemic symptoms.
DISCUSSION
Varicella zoster virus belongs to the herpesvirus family, which also encompasses cytomegalovirus, Epstein-Barr virus, herpes simplex viruses, and human herpesviruses. Herpesviruses can cause asymptomatic infection in some hosts. They can also become latent and later reactive and cause symptoms. The elderly and immunocompromised are more at risk for this reactivation. Primary VZV is known as chickenpox, a contagious febrile illness that presents primarily in childhood with nonspecific symptoms and a vesicular rash. Modern vaccines have greatly reduced the incidence of chickenpox.
Secondary VZV is known as shingles or herpes zoster. This occurs in patients who have had chickenpox in the past, although some patients may have had an asymptomatic and thus unknown primary VZV infection. Thirty percent of people in the United States experience shingles. Risk factors include immunosuppression related to medications, HIV, autoimmune diseases, age greater than 50, and female sex. Disseminated zoster is characterized by involvement of three or more dermatomes.
Varicella is spread through both airborne droplets and direct contact with the lesions. Diagnosis is typically based on symptoms and the presence of the classic vesicular rash, usually in a dermatomal distribution (see Fig 1). Patients are contagious forty-eight hours prior to the appearance of lesions until new lesions have stopped forming and all existing lesions have crusted over. Shingles can cause severe neuropathic pain, even prior to the presence of the characteristic rash. Systemic symptoms are uncommon but can include malaise, headache, fever. For those with lesions in the V1 distribution and/or on the tip of the nose, ophthalmologic evaluation is advised to assess for herpes zoster ophthalmicus, which can be vision threatening. VZV pneumonia, although overall rare, is the most common cause of death in adults with VZV. Pregnant patients are at risk of severe pneumonia and of pregnancy loss. Other uncommon complications include VZV encephalitis, Ramsay Hunt syndrome, acute retinal necrosis, aseptic meningitis, and cerebellar ataxia.
Figure 1: Herpes zoster rash (CoreEM [6])
All patients should receive analgesia. Acetaminophen and nonsteroid anti-inflammatory drugs are the mainstays of pain control. Opioids can be given for severe pain. Gabapentin, pregabalin, and tricyclic antidepressants have mixed evidence for pain control in acute zoster but can be considered as adjuncts. Glucocorticoids are controversial and not routinely recommended, except in cases of ocular or CNS involvement.
To treat herpes zoster, oral valacyclovir is recommended for symptom onset less than three days, if new lesions are still occurring, and/or for immunocompromised patients. These patients can typically be discharged with primary care follow up. Admission and intravenous acyclovir are recommended in disseminated zoster, VZV pneumonia, VZV in pregnant patients, VZV meningitis or encephalitis, and VZV in immunocompromised patients. Renal function must be closely monitored. Post-herpetic neuralgia is common and can be debilitating. Treatment is gabapentin or pregabalin.
The shingles vaccine is recommended in adults aged sixty. It can prevent or lessen the severity of herpes zoster and post-herpetic neuralgic.
CASE RESOLUTION
While admitted, the patient was continued on intravenous acyclovir. Infectious disease and dermatology were consulted. These services felt her presentation was most consistent with disseminated shingles, given her report of prodromal pain with subsequent presentation of lesions, immunosuppressed status, and that she had not had the shingles vaccine. Dermatology also felt the intact lesions exhibited classic “dew drop on a petal” morphology, in keeping with a classic disseminated zoster rash. A CT abdomen pelvis was unremarkable. VZV PCR swab of the lesions came back positive, whereas HSV PCR of the lesions came back as negative. She remained hemodynamically stable and did not show any signs of progressive organ failure. Repeat chest x-ray later in her course did not show signs of pulmonary involvement. She was taken off precautions when new lesions ceased to develop and when all current lesions were crusted over. She was transitioned to oral valacyclovir to complete a fourteen-day course.
TAKE-AWAYS
Shingles (herpes zoster) is a secondary VZV infection, most common in the elderly and/or immunocompromised.
Infection is typically localized and self-limiting.
Disseminated infection is characterized by involvement of 3 or more dermatomes.
Treatment is oral valacyclovir for uncomplicated infections, with admission and IV acyclovir recommended for disseminated infections or in those who are pregnant or immunocompromised.
Author: Alexandra Pusateri, MD, is a current second-year resident at Brown Emergency Medicine Residency.
Faculty Reviewer: Michelle Myles, MD, is an assistant professor and clinician educator at Brown Emergency Medicine.
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