Background
Varicella-zoster virus (VZV) is the cause of chickenpox and herpes zoster (also called shingles). Chickenpox follows initial exposure to the virus and is typically a relatively mild, self-limited childhood illness with a characteristic exanthem.
Approximately 1 per 4000 children develops VZV encephalitis, an acute neurologic disorder with potentially severe complications. In addition, immunocompromised children (eg, those receiving chemotherapy for leukemia or those with advanced HIV infection) can develop disseminated VZV infection, a potentially fatal complication.
After primary infection, VZV remains dormant in sensory nerve roots for life. Upon reactivation, the virus migrates down the sensory nerve to the skin, causing the characteristic painful dermatomal rash. After resolution, many individuals continue to experience pain in the distribution of the rash (postherpetic neuralgia). In addition, reactivation of VZV infection can cause a spectrum of atypical presentations, ranging from self-limited radicular pain without rash to spinal cord disease with weakness.
Pathophysiology
The host immunologic mechanisms suppress replication of the virus. Reactivation can occur if host immune mechanisms are compromised. This may be caused by medications, illness, malnutrition, or by the natural decline in immune function with aging. Upon reactivation, the virus migrates along sensory nerves and produces sensory loss, pain, and other neurologic complications. If motor nerve roots are also involved, weakness can develop in addition to sensory changes. Leptomeningeal involvement is rare but may develop when the ophthalmic branch of the trigeminal nerve is involved.
Epidemiology
Frequency
United States
The rate of occurrence is about 5 persons per 1000 population. Immunosuppression increases this risk. The risk of postherpetic neuralgia increases with age. Approximately 50% of patients older than 60 years may have temporary or prolonged pain syndrome.
The frequency of VZV infection may decrease as the immunized children become adults.
International
VZV infection occurs with the same frequency in the United States and internationally.
Mortality/Morbidity
- Severe pain and insomnia are most bothersome to patients. About 95% of patients with zoster experience severe pain during the illness.
- Other presentations of zoster, including ocular (keratitis) and spinal cord (myelitis) presentations, may result in additional morbidity.
- Bacterial superinfection (impetiginization) of vesicular skin lesions can occur.
Race
The vesicular eruption of VZV infection may be more difficult to diagnose in patients with darker skin.
Sex
VZV infection occurs with equal frequency in males and females.
Age
- After primary infection, zoster can occur at any age. However, the risk of zoster increases with age.
- The risk of postherpetic neuralgia also increases with advancing age.
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