Herpes zoster, also called shingles, is characterized by a unilateral vesicular eruption within a dermatome, often associated with severe pain. The dermatomes from T3 to L3 are most frequently involved. If the ophthalmic branch of the trigeminal nerve is involved, zoster ophthalmicus results. The factors responsible for the reactivation of VZV are not known. In children reactivation is usually benign, whereas in adults it can be debilitating. The continuum of pain from onset to resolution is known as zoster-associated pain. The onset of disease is heralded by pain within the dermatome that may precede lesions by 48 to 72 h; an erythematous maculopapular rash evolves rapidly into vesicular lesions. In the normal host, these lesions may remain few in number and continue to form only for a period of 3 to 5 days. The total duration of disease is generally between 7 and 10 days; however, it may take as long as 2 to 4 weeks for the skin to return to normal. In a few patients, characteristic localization of pain to a dermatome with serologic evidence of herpes zoster has been reported in the absence of skin lesions. When branches of the trigeminal nerve are involved, lesions may appear on the face, in the mouth, in the eye, or on the tongue. In the Ramsay Hunt syndrome, pain and vesicles appear in the external auditory canal, and patients lose their sense of taste in the anterior two-thirds of the tongue while developing ipsilateral facial palsy. The geniculate ganglion of the sensory branch of the facial nerve is involved.
The most debilitating complication of herpes zoster, in both the normal and the immunocompromised host, is pain associated with acute neuritis and postherpetic neuralgia. Postherpetic neuralgia is uncommon in young individuals; however, at least 50% of patients over age 50 with zoster report some degree of pain in the involved dermatome months after the resolution of cutaneous disease. Changes in sensation in the dermatome, resulting in either hypo- or hyperesthesia, are common.
CNS involvement may follow localized herpes zoster. Many patients without signs of meningeal irritation have CSF pleocytosis and moderately elevated levels of CSF protein. Symptomatic meningoencephalitis is characterized by headache, fever, photophobia, meningitis, and vomiting. A rare manifestation of CNS involvement is granulomatous angiitis with contralateral hemiplegia, which can be diagnosed by cerebral arteriography. Other neurologic manifestations include transverse myelitis with or without motor paralysis.
Like chickenpox, herpes zoster is more severe in the immunocompromised host than in the normal individual. Lesions continue to form for over a week, and scabbing is not complete in most cases until 3 weeks into the illness. Patients with Hodgkin's disease and non-Hodgkin's lymphoma are at greatest risk for progressive herpes zoster. Cutaneous dissemination develops in about 40% of these patients. Among patients with cutaneous dissemination, the risk of pneumonitis, meningoencephalitis, hepatitis, and other serious complications is increased by 5 to 10%. However, even in immunocompromised patients, disseminated zoster is rarely fatal.
Patients who have received a bone marrow transplant are at particularly high risk of VZV infection. Thirty percent of cases of posttransplantation VZV infection occur within 1 year (50% of these within 9 months); 45% of the patients involved have cutaneous or visceral dissemination. The mortality rate in this situation is 10%. Postherpetic neuralgia, scarring, and bacterial superinfection are especially frequent in VZV infections occurring within 9 months of transplantation. Among infected patients, concomitant graft-versus-host disease increases the chance of dissemination and/or death.
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