2.1.1 Varicella and Herpes Zoster
ICD-11
1E90
Synonyms
Chickenpox; shingles.
Definition
Varicella-zoster virus (VZV) causes a primary infection usually in childhood (varicella, chickenpox) and may later reactivate in a nerve (herpes zoster, shingles).
Epidemiology
Chickenpox is a highly contagious disease (by respiratory or direct contact). Usually affects children between 2 and 10 years of age. Endemic outbreaks in spring and autumn. The incubation period for varicella is between 10-21 days and it is highly contagious, via direct skin contact, or contact with aerosolized particles from the vesicles or respiratory secretions. It is a highly prevalent disease (90% of adults have suffered) and gives permanent immunity.
The incidence of herpes zoster ranges between 1.3 and 4 cases per 1000 individuals / year. More than two thirds of cases occur in individuals over 50 years. 10% of cases of shingles occur in individuals with risk factors (cancer, previous trauma, etc.). Its incidence in immunosuppressed subjects is much higher than the general population.
Aetiology & Pathogenesis
None.
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VZV is transmitted by the airborne route via coughs, sneezes and sometimes direct contact with the skin of infected individuals. See below for the pathogenesis of herpes zoster.
Signs & Symptoms
Varicella: Incubation period: 1-2 weeks, highly contagious. Clinical features: papulo-vesiculo-pustular exanthem with lesions in varying stages of development.
Herpes zoster: Pathogenesis: viruses persist in the spinal and cranial ganglia (Gasserian ganglion, geniculate ganglion). Exacerbating factors: immunosuppression, trauma, sunlight, severe illnesses.
Clinical features: segmental lesion not crossing the midline with burning, pain, erythema, blisters, necrosis, scars, post-herpetic neuralgia. Herpes zoster haemorrhagicus, gangrenosus- more severe forms; generalised- widespread, resembles varicella with aberrant blisters.
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The rash is a papulo-vesiculo-pustular exanthem. A helpful diagnostic feature is the presence of lesions in varying stages of development. The patient generally feels unwell, may be pyrexial and is typically itchy.
VZV may be harboured in the spinal and cranial ganglia (Gasserian ganglion, geniculate ganglion) and has a potential to reactivate, causing herpes zoster. Factors which may increase the chance of reactivation include immunosuppression, trauma, sunlight and other severe illnesses. Clinical features are usually typical, with a segmental lesion not crossing the midline. Clinical features may include burning sensations, pain, erythema, blisters, necrosis, scars and post-herpetic neuralgia. Herpes zoster haemorrhagicus, gangrenosus is a severe form with generalised disease resembling varicella with aberrant blisters. Often in the pre-eruptive phase depending on the localization it may mimic symptoms of cardiac infarction, gallbladder or renal attacks, colon pain including ileus symptoms or lumbago or migraine attacks
Localisation
Varicella: head and mouth (gums) are often involved but palms and soles are usually spared.
Zoster: in affected dermatome.
Variants: Ophthalmic herpes zoster: 1st trigeminal branch (V1 nerve) sometimes with nasociliary branch; be aware of need for urgent opthalmology review, keratitis. Herpes zoster 2nd or 3rd trigeminal branch. Aural herpes zoster (VIIIth nerve); acoustic nerve and potential facial paralysis.
Classification
According to dermatome.
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Nil.
Laboratory & other workups
Only required if severe involvement or other relevant clinical question. Viral swabs may confirm the diagnosis days later. Immediate confirmation when required via Tzanck smear, PCR of vesicle fluid or antigen test.
Dermatopathology
Not usually required. Intraepithelial blisters and destruction of sebaceous glands are indistinguishable from other viral infections.
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Skin biopsies are almost never required to make the diagnosis. Where taken, intraepithelial apoptosis, necrosis and vacuolated keratinocytes followed by blisters and destruction of sebaceous glands are indistinguishable from other viral infections.
Course
Usually a self-limiting disease. Varicella is commonly mild in young children but may be disseminated and severe in adults (especially in pregnancy), rarely leading to multi-organ failure.
Complications
Varicella: fever, malaise, pneumonia, scarring -especially when lesions display secondary infection. Fetal malformations may occur after infection in the 1st trimester; neonatal varicella when infection occurs in the 3rd trimester.
Herpes zoster: dissemination, superadded infection, keratitis and postherpetic neuralgia.
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Varicella may rarely cause pneumonia. Scarring of the skin is common following infection, especially when lesions display secondary infection. Fetal malformations may occur after infection in the 1st trimester; neonatal varicella may occur when infection of the mother is in the 3rd trimester. Herpes zoster may disseminate showing the typical chicken pox lesions. Superadded infection may occur, especially after excoriation. Keratitis is relatively rare. Postherpetic neuralgia may cause long-lasting pain for months or years.
Diagnosis
Usually clinical.
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The diagnosis is usually clinical, but clinical features of early herpes zoster can be misleading and the early features may be mistaken for insect bites or other skin disease.
Differential Diagnosis
- Varicella: insect bites, Coxsackie virus infection, generalized herpes zoster, eczema herpeticum, and other viral exanthems.
- Herpes zoster: insect bites; herpes simplex; pain from other causes.
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The differential diagnosis for varicella includes insect bites, Coxsackie virus infection, generalized herpes zoster, eczema herpeticum, and other viral exanthems. For herpes zoster, the differential diagnosis includes insect bites, herpes simplex or pain from other causes. In the face initial erysipelas or acute phase of contact dermatitis has to be considered as well as on the lower leg.
Prevention & Therapy
Prevention of herpes zoster reactivation: immunization.
Topical: antiseptics (e.g. chlorhexidine 1% lotion).
Systemic: antiviral agents (e.g. aciclovir). Indications for systemic treatment: patients> 50 years, disease present less than 3 days, tendency towards generalization, eye involvement (1st branch of trigeminal nerve), immunodeficiency. Analgesics as required (NSAIDs, but acetyl salicylic acid contraindicated re Reyes syndrome) and, in special cases, corticosteroids.
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Prevention of herpes zoster reactivation can be achieved with immunization but different European countries have different recommendations. Some recommend vaccination in the second year of life; some suggest vaccination only in those not having had chickenpox by the age of 12-14 and some only recommend vaccination in special circumstances. For treatment of active varicella, topical agents include astringents and antiseptics (e.g. chlorhexidine 1% lotion). Systemic treatment options should be considered in certain cases (2) and include antiviral agents (e.g. aciclovir and valaciclovir). Indications for systemic treatment are patients> 50 years, disease present less than 3 days, tendency towards generalization, eye involvement (1st branch of trigeminal nerve) or immunodeficiency. Analgesics or anti-pyretics should be administered as required (NSAIDs, but acetyl salicylic acid is contraindicated because it may be associated with Reyes syndrome) and, in special cases, corticosteroids.
Special
Ask about contacts of the index patient (pregnant individuals, children, immunocompromised).
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