Specific History

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Onset

Zoster attacks may occur in children and young adults, but are quite rare. The incidence starts to climb during the fifth decade of life and peaks in the seventh and eighth decades. Severe or prolonged attacks, especially in young persons, should raise concern about concomitant illness and immune status. An attack usually confers lifelong immunity in an otherwise healthy person.

Evolution of Disease Process

Most cases of herpes zoster present with pain that is variously described as shock-like or a continuous burning sensation with hyperalgesia. Other patients experience less intense but equally uncomfortable crawling or pruritic parasthesias, and find that even fabric touching the area is intolerable. Within 2 or 3 days, and rarely as long as a week, skin lesions develop within the anatomic area of the involved nerve segment. These lesions, like those of herpes simplex virus (HSV), consist of tightly grouped vesicles on an ery-thematous, urticarial base. The lesions are usually more extensive than those of HSV and may be continuous or, more often, exhibit skip areas within the neurologic segment. Mild constitutional symptoms of fatigue and lassitude may precede the skin lesions, but fever is rare.

Uncomplicated zoster in children usually is mild and often painless. It can run its entire course in 2 weeks or less, and normally clears without sequelae. In young adults, the average course is 2 to 3 weeks long, pain is mild to moderate, and sequelae are rare. Elderly, debilitated, or immunologically compromised patients often have a course of 3 to 4 weeks or longer, and are more prone to complications. In uncomplicated cases, the involution of the skin lesions and resolution of sensory symptoms parallel each other. On occasion, painful zoster may occur without skin lesions. This variant is called zoster sine eruptione, and has been responsible for exploratory laparotomies when abdominal segments and visceral nerve branches were involved. Cutaneous lesions without accompanying pain are referred to as zoster sine neuralgia.

Although most cases are unilateral, bilateral zoster does occur. The following anatomic locations are most frequently involved:

Thoracic dermatomes 53%

Cervical dermatomes 20%

Trigeminal nerve 15%

Lumbosacral dermatomes 11%

Special forms of herpes zoster include the following:

Trigeminal zoster: Any of the three divisions of the fifth cranial nerve may be affected (see Fig. 3). On rare occasions more than one branch is simultaneously involved. The ophthalmic division is most often attacked, and this site increases in frequency with advancing age. Lesions may occur on the eyelids, forehead, and anterior scalp, and if the nasociliary branches are affected, severe ocular sequelae may result. Zoster lesions on the distal nasal sidewall or nasal tip (Hutchinson's sign; see Photo 15) should immediately raise concern about the eye. Uveitis, keratitis, conjunctivitis, ocular muscle paralysis, scle-ritis, retinal vascular occlusion, and paralysis of the pupil may follow. Any ocular symptoms should prompt an immediate ophthalmologic examination. Consultation is recommended with severe cases, even in the absence of eye symptoms.

Involvement of the maxillary division is associated with vesicles on the tonsil and uvula. Mandibular zoster causes lesions on the buccal mucosa, floor of the mouth, and the anterior part of the tongue. Nerve damage may lead later to loss of teeth.

Ramsay-Hunt syndrome: The classic description includes the following triad of findings: (1) zoster lesions on the pinna (see Photo 16), meatus, and canal or tympanic membrane of one ear, (2) severe ear pain, and (3) an ipsilateral facial nerve palsy (see Photo 17). Vestibular symptoms and sensorineural hearing loss may also occur. The facial paralysis is usually complete and the recovery rate is low. Taste and lacrimation may also be affected.

OPHTHALMIC DIVISION

OPHTHALMIC DIVISION

Figure 3: Major division of the trigeminal nerve.

Sacral zoster with motor involvement: Though we think of zoster as a sensory nerve problem, motor fibers are involved in about 5% of cases. When sacral segments are involved, fecal retention or bladder disturbances may occur. Hemorrhagic cystitis and incontinence have been reported. In one case, an elderly man was hospitalized with unexplained lower abdominal pain and acute urinary retention. Studies revealed an adynamic bladder. On the fifth hospital day, zoster lesions became evident in a sacral dermatome distribution (see Photo 18 for an example of sacral zoster).

Complications of herpes zoster include the following:

Generalized zoster: In addition to the problems associated with the special forms reviewed above, one of the most serious complications is generalized herpes zoster. Many patients will develop a few scattered lesions that are out of the primary neurologic segment. When extensive lesions occur along with fever and systemic toxicity, however, it is an indication of general viremia. Fatalities can occur. Elderly, debilitated, and immunologically compromised patients are most often affected. Early routine administration of antiviral agents for zoster should reduce the sequelae of this dread complication (see Photo 19).

Postherpetic neuralgia (PHN): Acute neuritis associated with a segmental zoster attack is uncommon in children and young adults who are otherwise in good health. The incidence of severe neuritis increases after age 40, as does the incidence of postherpetic neuralgia. This complication (persisting pain and/or altered sensation in the affected nerve segments after healing of the skin lesions) occurs in more than 50% of cases in some series reported. It is the most common complication of herpes zoster. The incidence peaks in patients in their sixth and seventh decades, probably due to more severe attacks and lowered capacity to regenerate after nerve injury. Most patients without long-term neurologic sequelae note clearing of the neurologic symptoms before or concurrently with healing of the skin lesions. Pain or altered nerve function persisting more than 30 days after the onset of skin lesions is considered PHN. Of those with persisting pain or altered sensation, a large number gradually improve and clear over several months. Some patients have permanent symptoms. Lancinating pain, hyperalgesia, and crawling dysesthesias are most common. These symptoms may be severe enough to be physically disabling or may be a source of chronic depression.

Other rare complications: Encephalitis, myelitis, cranial/peripheral nerve palsies, delayed contralateral hemiparesis, and acute retinal necrosis have been associated with herpes zoster.

Evolution of Skin Lesions

Zoster lesions begin with an urticarial plaque that is at first bright-red in color. Abortive lesions may simply regress. In most instances, however, tightly grouped papules develop within several hours to a few days. These rapidly evolve into vesicles and then into flaccid pustules. In uncomplicated cases, the eruption peaks in 4 to 5 days, stabilizes, then heals over a period of 2 to 3 weeks. Lesions should remain well localized to the involved nerve segment. Regional nodes may be reactive. As the pustules dry, crusts form, which are shed over several days. Even uncomplicated zoster lesions may leave long-standing postinflammatory hyperpigmentation. Hemorrhagic lesions are more frequent in patients with underlying disease (see Photo 20). Elderly and debilitated patients are prone to deep, slow-healing, necrotic, or gangrenous lesions that leave substantial scarring and pigmentation (see Photo 15).

Provoking Factors

Immunosuppression, whether iatrogenic or secondary to disease, predisposes to herpes zoster. Hemorrhagic zoster lesions or zoster of unusual severity in a young host should raise suspicion of underlying disease such as lymphoma, hematologic malignancies, or HIV disease.

Physical trauma to infected sensory ganglia, and occasionally to peripheral nerves, can trigger attacks. Radiation therapy of solid tumors and spinal manipulation are among the other common causes.

Self-Medication

Self-treatment is seldom a problem in herpes zoster.

Supplemental Review From General History

Whenever zoster is atypical, generalized, hemorrhagic, gangrenous, or unusually prolonged, a careful general history and exam should be done for evidence of an underlying lymphoma, leukemia, solid tumor, or possible HIV disease. In many instances, the atypical course can be explained by existing treatments.

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