History
The following are the most common presenting symptoms of varicella:
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Low-grade fever preceding skin manifestations by 1-2 days
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Complaints of abdominal pain by some children
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Pleomorphic rash, usually starting on the head and trunk and spreading to the rest of the body
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Typically, complaints of intense pruritus
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Headache
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Malaise
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Anorexia
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Cough and coryza
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Sore throat
Children with eczema or dermatitis may have severe skin manifestations during varicella.
The history should elicit if a recent outbreak of chickenpox in the community has occurred and if any exposure to varicella at school, daycare, or among family members has occurred. Most patients have a history of exposure in one or more of these three settings. It should also be noted whether the child has previously received varicella vaccine or if the child is immunocompromised (including recent systemic steroid use) to help guide management.
Immunocompromised children often have severe and complicated varicella, and their mortality rate is higher than that in immunocompetent children. Such children are at high risk for developing progressive varicella with multiple organ involvement. These children may have prolonged high fever, prolonged extensive rashes, and hepatitis.
The following categories of patients should be considered immunocompromised:
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Children with any malignancy
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Children on cancer chemotherapy
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Children undergoing high-dose corticosteroid therapy
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Children with congenital cellular immunodeficiencies
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Children on immunosuppressive therapy
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Children with HIV infection
Ask parents whether their child had chickenpox previously. Recently, the validity of reported varicella history as a marker for immunity among unvaccinated individuals has come into question. A recent study recommended that a reported history is no longer highly predictive of seropositivity, suggesting that universal vaccination regardless of history may be prudent. [23]
Physical Examination
Rash and fever are the typical findings during physical examination in pediatric patients with varicella. An ill appearance should raise concern for pulmonary or neurologic complications or serious bacterial superinfection. (See Complications.)
Examination of rash
The diagnosis of varicella is made upon observation of the characteristic chickenpox rash. This rash appears in crops. Skin lesions initially appear on the face and trunk, beginning as red macules and progressing over 12-14 days to become papular, vesicular, pustular, and finally crusted. New lesions continue to erupt for 3-5 days. Lesions usually crust by 6 days (range 2-12 d), and completely heal by 16 days (range 7-34 d). Prolonged eruption of new lesions or delayed crusting and healing can occur with impaired cellular immunity.
An otherwise healthy child usually has 250-500 lesions but may have as few as 10 or as many as 1500. The lesions predominate in central skin areas and proximal upper extremities with relative sparing of distal and lower extremities but spread to other skin areas. Some lesions may appear in the oropharynx. Eye lesions are rare.
Each lesion starts as a red macule and passes through stages of papule, vesicle, pustule, and crust. The vesicle on a lesion’s erythematous base leads to its description as a pearl or dewdrop on a rose petal. Vesicles may occur on mucous membranes and break down to form shallow aphthous ulcers. Vesicles can be hemorrhagic. Redness or swelling around a lesion should lead to suspicion of bacterial superinfection. Dermatomal distribution of lesions is characteristic of reactivation rather than primary infection.
The hallmark of the disease is the simultaneous presence of different stages of the rash.
Assessment of fever
Fever is usually low grade (100-102°F) but may be as high as 106°F. In otherwise healthy children, fever typically subsides within 4 days. Prolonged fever should prompt suspicion of complication or immunodeficiency. Although tachypnea may be seen with fever alone, respiratory distress might represent pneumonitis.
In Utero Varicella Infections
Outcomes of in utero varicella infections vary based on the timing of the infection.
The Royal College of Obstetricians and Gynaecologists released revised guidelines for treating chickenpox in pregnancy, summarized below. [24, 25]
Clinicians should ask women presenting for antenatal care about previous chicken pox or shingles infection.
Pregnant women who have not had chicken pox, or who are known to be seronegative for chicken pox, should avoid contact with persons who have chicken pox or shingles and should promptly inform their clinician of potential exposure.
Clinicians should confirm potential exposure by careful history to confirm the significance of the contact and the susceptibility of the patient, as well as by blood test to determine VZV immunity or nonimmunity.
Pregnant women may need a second dose of varicella-zoster immunoglobulin if there is further exposure and 3 weeks have elapsed since the last dose.
Pregnant women who develop the characteristic rash should immediately inform their clinician, and they should be isolated from other pregnant women and neonates until the lesions have crusted over (usually about 5 days after rash onset).
Symptomatic treatment and hygiene are helpful to prevent secondary bacterial infection.
Aciclovir is not licensed for use in pregnancy. Clinicians should advise their patients of the risks and benefits.
Clinicians should consider hospital assessment of women at high risk for severe or complicated chicken pox, regardless of clinical status.
Clinicians should refer pregnant women who develop chicken pox to a fetal medicine specialist, virologist, and neonatologist for decision regarding treatment.
Clinicians should individualize the timing and mode of delivery of the pregnant woman with chicken pox.
Women with chicken pox should breast-feed if they so desire and are in sufficiently good health.
Congenital varicella syndrome
Congenital varicella syndrome occurs in 2% of children born to women who develop varicella during the first or second trimester of pregnancy. [26] It manifests as intrauterine growth retardation, microcephaly, cortical atrophy, limb hypoplasia, microphthalmia, cataracts, chorioretinitis, and cutaneous scarring. Fetal injury risk is unrelated to the severity of disease in the mother. Zoster exposure during pregnancy has not been associated with fetal injury.
Infantile zoster
Infantile zoster is caused by maternal varicella infection after 20 weeks’ gestation. It usually manifests within the first year of life and commonly involves the thoracic dermatomes. [27]
Neonatal varicella
Neonatal varicella can be a serious illness, depending on the timing of maternal varicella and delivery. After the initial viremia, the mother develops antibodies against the varicella virus. The severity of the disease in the newborn depends on whether transplacental passage includes only the virus or includes both the virus and the antibodies.
If the mother develops varicella within 5 days before or 2 days after delivery, the baby is exposed to the secondary viremia of the mother. The baby transplacentally acquires the virus but acquires no protective antibodies because of insufficient time for antibodies to develop in the mother. In these babies, neonatal varicella is likely to be severe and disseminated. Hemorrhagic lesions of the liver and lungs characterize this potentially fatal disease.
Onset of maternal varicella more than 5 days ante partum provides the mother sufficient time to manufacture and pass on antibodies along with the virus. Full-term neonates of these women usually have mild varicella because of the attenuating effect of the transplacentally acquired antibodies.
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The pleomorphic rash characteristic of varicella. Papules, vesicles, and pustules are concurrently present.
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Papulovesicular lesions on arm in varying stages of healing in this infant with varicella. Photograph courtesy of Susan Feigelman, MD.
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- Overview
- Presentation
- DDx
- Workup
- Treatment
- Approach Considerations
- Supportive Therapy
- Antiviral Therapy
- Varicella-Zoster Immune Globulin Therapy
- Treatment of Neonatal Varicella
- Treatment of Secondary Bacterial Infections
- Varicella Vaccination
- Admission to Intensive Care Unit
- Transfer to Tertiary Care Facility
- Dietary Measures
- Restriction of Activity
- Consultations
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- Questions & Answers
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