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Author: Parang N Mehta, MD, Consulting Staff, Department of Pediatrics, Mehta Contact Derm
Hospital, Surat, India
Coauthor(s): Archana Chatterjee, MD, Assistant Professor, Department of Enteroviral
Pediatrics, Division of Pediatric Infectious Disease, Creighton University Infections
Herpes Simpl
Editor(s): Leonard R Krilov, MD, Chief of Pediatric Infectious Diseases, Virus Infection
Department of Pediatrics, Winthrop University Hospital; Robert Konop, PharmD,
Clinical Assistant Professor, Department of Pharmacy, Section of Clinical Impetigo
Pharmacology, University of Minnesota; Leslie L Barton, MD, Professor, Program
Director, Department of Pediatrics, University of Arizona School of Medicine; Urticaria
Robert W Tolan, Jr, MD, Chief of Pediatric Infectious Diseases, Chairman,
Department of Pediatrics, Capital Health System; Clinical Associate Professor of
Pediatrics, Drexel University College of Medicine; and Russell Steele, MD, Continuin
Department Head and Vice-Chair, Professor, Department of Pediatrics, Division of Educatio
Infectious Diseases, Louisiana State University and New Orleans Children's
CME available
Hospital this topic. Clic
INTRODUCTION Section 2 of 11 here to take th
CME.
Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Pictures
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Patient Educ
Click here
Background: Varicella, commonly known in the United States as chickenpox, is patient educa
caused by the varicella-zoster virus. The disease generally is regarded as a mild self-
limiting viral illness with occasional complications. Before vaccination for varicella
became widespread in the United States, this disease caused as many as 100
deaths annually.
Even today, varicella is not totally benign. A recent study suggests that nearly 1 in 50
cases of varicella are associated with complications; among the most dreaded are
varicella pneumonia and encephalitis, both associated with a high mortality rate. In
addition, significant concerns have recently been raised about the association of
varicella with severe invasive group A streptococcal disease.
The United States adopted universal vaccination against varicella in 1995, which
reduced morbidity and mortality from this disease. Children who are not vaccinated,
for various reasons, remain susceptible. Children with varicella expose adult contacts
in households, schools, and daycare centers to the risk of severe, even fatal,
disease. Varicella is common and highly contagious and affects nearly all susceptible
children before adolescence.
Household transmission rates are 80-90%. Second cases within the household often
are more severe. School or daycare center contact is associated with lower but still
significant transmission rates. Susceptible children rarely acquire the disease by
contact with adults with zoster. Maximum transmission occurs during late winter and
spring.
After a week, a secondary viremia disseminates the virus to the viscera and skin,
eliciting the typical skin lesions. This viremia also spreads the virus to respiratory
sites and is responsible for the contagion of varicella before the appearance of the
rash. Infection of the CNS or liver also occurs at this time.
Frequency:
In the US: Before varicella vaccine use became widespread, 4 million cases
of chickenpox were reported annually. The disease was responsible for 11,000
hospitalizations each year and approximately 50-100 deaths.
Internationally: Varicella affects nearly all children who do not have immunity.
Annual incidence is estimated at 80-90 million cases.
Mortality/Morbidity: In otherwise healthy children aged 1-14 years, the mortality rate
is estimated at 2 deaths per 100,000 cases.
Most deaths in the United States before universal vaccination were from
associated encephalitis, pneumonia, secondary bacterial infection, and Reye
syndrome.
Age: Maximum incidence of varicella is in children aged 1-6 years. Persons older
than 14 years account for 10% of varicella cases.
CLINICAL Section 3 of 11
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History:
Exposure
Prodrome
o Rash, usually starting on the head and trunk and spreading to the rest
of the body
o Headache
o Malaise
o Anorexia
o Sore throat
Physical:
Rash
o An otherwise healthy child usually has 250-500 lesions but may have
as few as 10 or as many as 1500.
o Lesions usually crust by 6 days (2- to 12-d range), and heal completely
by 16 days (7- to 34-d range).
o Prolonged eruption of new lesions or delayed crusting and healing can
occur with impaired cellular immunity.
Fever
Outcomes of in utero varicella infections vary, based upon the timing of the
infection as follows:
o Infantile zoster
Infantile zoster usually manifests within the first year.
The cause is maternal varicella infection after the 20th week of
gestation.
Infantile zoster commonly involves the thoracic dermatomes.
o Neonatal varicella
Neonatal varicella can be a serious illness, depending upon the
timing of maternal varicella and delivery.
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 acquires the virus transplacentally but
acquires no protective antibodies because of insufficient time for
antibodies to develop in the mother. In these circumstances,
neonatal varicella is likely to be severe and disseminated.
Prophylaxis or treatment is required with varicella-zoster immune
globulin (VZIG) and acyclovir. Without these drugs, mortality
rates may be as high as 30%. The primary causes of death are
severe pneumonia and fulminant hepatitis.
Onset of maternal varicella more than 5 days antepartum
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. Treatment with
VZIG is not recommended in such cases, but acyclovir may be
used, depending on individual circumstances.
Causes:
Varicella is highly contagious; secondary attack rates range from 80-90% for
household contacts.
Transmission
o Papules and vesicles, but not the crusts, have high populations of the
virus.
o Malignancy: All children with cancer have an increased risk for severe
varicella. The risk is highest for children with leukemia. Almost 30% of
patients who are immunocompromised and who have leukemia have
visceral dissemination of varicella; 7% may die.
DIFFERENTIALS Section 4 of 11
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Contact Dermatitis
Enteroviral Infections
Herpes Simplex Virus Infection
Impetigo
Urticaria
Drug reactions
Insect bites
Smallpox
WORKUP Section 5 of 11
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Lab Studies:
Laboratory studies are unnecessary for diagnosis because varicella is obvious clinically.
Most children with varicella have leukopenia in the first 3 days, followed by leukocytosis.
Marked leukocytosis may indicate a secondary bacterial infection, but this is not a dependable
Most children with significant secondary bacterial infections do not have leucocytosis.
Immunohistochemical staining of skin lesion scrapings can confirm varicella.
o The procedure is useful for high-risk patients who require rapid confirmation.
o A Tzanck smear involves scraping the base of the lesions, then staining the scrapings
demonstrate multinucleated giant cells. This finding, however, is not sufficiently sensitiv
specific for varicella and should be replaced by the more specific immunohistochemica
staining of such scrapings, if available.
Serologic studies
o Serology mainly is used to confirm past infection to assess a patient's susceptibility sta
This helps determine preventive treatment requirements for an adolescent or adult who
been exposed to varicella.
o Among the many serologic studies, the most sensitive are the indirect fluorescent antib
(IFA), fluorescent antibody to membrane antigen (FAMA), neutralization test (NT), and
radioimmunoassay (RIA). These time-consuming tests require specialized equipment t
renders them unsuitable for routine use.
Imaging Studies:
Chest x-ray
o Children with high temperatures and respiratory signs should have a chest x-ray to con
exclude pneumonia.
o Chest x-ray findings may be normal or may show diffuse bilateral nodular infiltrates in p
varicella pneumonia. X-rays also may detect focal infiltrates suggestive of secondary b
pneumonia.
Other Tests:
Lumbar puncture
o Children with neurological signs should have their cerebrospinal fluid (CSF) examined.
o The CSF of patients with varicella encephalitis may have few or as many as a hundred
that are polymorphonuclear or mononuclear, depending on the timing of the lumbar pu
TREATMENT Section 6 of 11
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Medical Care:
Discourage scratching to avoid scarring. Trimming the child's fingernails and having the child
mittens while sleeping may reduce scratching.
Consultations:
Children who develop severe and life-threatening varicella complications may require hospita
in an ICU.
Diet:
Some children with varicella have reduced appetite and should be encouraged to take sufficie
fluids to maintain hydration. Adequate hydration is especially important if the child is receiving
acyclovir, as the drug can crystallize in the renal tubules if administered to dehydrated individu
Activity: No activity restrictions are needed for young children with uncomplicated varicella.
MEDICATION Section 7 of 11
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Drug Category: Antivirals -- Chickenpox is not always benign. In certain well-defined groups, varicella c
severe and even fatal. Antiviral drugs are recommended for adolescents, adults, and children on steroid or salic
therapy and for children who are otherwise immunocompromised. Acyclovir is the only adequately studied dru
this class.
Drug Category: Antipyretics -- Inhibits central synthesis and release of prostaglandins that mediate the e
endogenous pyrogens in the hypothalamus, thus, promotes the return of the set-point temperature to normal.
Fever usually is low grade but may be elevated. Acetaminophen probably is the safest drug to use for this purp
Salicylate usage for varicella is associated with Reye syndrome; therefore, never prescribe these agents. Nonst
anti-inflammatory drugs (NSAIDs) have been suspected of suppressing immune function and promoting infec
progress in patients infected with invasive group A streptococci.
Drug Category: Antihistamines -- May control pruritus by blocking effects of endogenous release of his
Pruritus can be severe in varicella, preventing sleep and possibly leading to scarring or secondary infection.
Nonsedating antihistaminics lack sufficient antipruritic action. The value of local preparations (eg, calamine,
antihistamines) is unproved. Topical antihistamines can cause significant sedation from absorption through inj
skin.
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o Seizures
o Difficulty walking
o Respiratory distress
o Cyanosis
Hospitalize and treat all newborns whose mothers developed varicella less than 5 days before
within 2 days after delivery.
Deterrence/Prevention:
Vaccination
o Varicella vaccine consists of live attenuated Oka strain varicella virus. The vaccine is s
highly immunogenic.
o Protection against varicella ranges from 71-100% and is likely to be long term. Breakth
varicella is mild when it occurs.
o The vaccine is effective when administered on or after the age of 1 year. Only a single
recommended for children younger than 13 years. For older children, the recommende
dosage is 2 doses separated by 4-8 weeks.
o Research study protocols allow varicella vaccine administration to patients with leukem
while they are in remission.
o Postexposure prophylaxis, if provided within 72 hours of contact, can prevent or attenu
disease in the exposed individual.
o VZIG reduces complications and the mortality rate of varicella, not its incidence.
Postexposure prophylaxis using varicella vaccine is preferred for immunologically norm
patients. VZIG is indicated for the following persons with significant exposure:
Newborns of mothers who acquired varicella 5 days before to 2 days after delive
Children with leukemia or lymphoma who have not been vaccinated and have n
varicella previously
Persons with HIV, AIDS, or other immunodeficiency disorders
Persons receiving drugs that suppress immune function (eg, systemic steroids)
Pregnant women
Immunocompromised individuals who have no reliable history of chickenpox
The American Association of Pediatrics (AAP) Red Book recommends excluding affected chil
from school until the sixth day of rash. This may not prevent spread of varicella because the c
infective before rash appears.
Complications:
o Varicella may predispose patients to bacterial infections. Skin lesion infections are com
and occur in 5-10% of children. Skin lesions provide a portal of entry for virulent organi
rapidly spreading cellulitis, septicemia, and other serious infections may occur.
o The most common infectious organisms are group A streptococci and Staphylococcus
aureus. Varicella places the patient at high risk for acquiring invasive group A streptoco
disease. In addition to toxic shock syndrome, group A streptococci may cause necrotiz
fasciitis, bacteremia, osteomyelitis, pyomyositis, gangrene, subgaleal abscess, arthritis
meningitis in patients with varicella.
o Staphylococcal species also cause severe infections in children with varicella. Staphylo
infections in these patients reportedly cause cellulitis, impetiginous pox infections,
staphylococcal scalded skin syndrome, toxic shock syndrome, pericarditis, and osteom
o Signs and symptoms of secondary bacterial infection can be indistinguishable from
uncomplicated varicella during the first 3-4 days.
o A high level of suspicion is necessary for early recognition and timely appropriate treatm
secondary infections.
o Suspect secondary infection when systemic manifestations do not improve in 3-4 days
fever returns or worsens, or the child's condition deteriorates after initial improvement.
o Suspicion of secondary bacterial infection should prompt early institution of empirical
antibiotic therapy until the results of culture studies become available.
o Neutrophilic leukocytosis and neutrophilia occur in only a few cases involving serious
bacterial infections.
o Investigations cannot be relied upon to diagnose or exclude bacterial infection.
CNS complications
o Acute postinfectious cerebellar ataxia is the most common CNS complication, with an
incidence of 1 case per 4000 patients with varicella.
Ataxia has sudden onset that usually occurs 2-3 weeks after the onset of varice
condition may persist for 2 months.
Manifestations may range from mild unsteadiness to complete inability to stand
walk, with accompanying incoordination and dysarthria. Manifestations are max
onset; a waxing and waning course suggests another diagnosis.
The sensorium is clear, even when the ataxia is profound.
The prognosis for patients with ataxia is good, but a few children may have resid
ataxia, incoordination, or dysarthria.
o Encephalitis occurs in 1.7 patients per 100,000 cases of varicella among otherwise hea
children aged 1-14 years.
The disease manifests during acute varicella a few days after rash onset. Letha
drowsiness, and confusion are the usual presenting symptoms.
Some children may have seizures, and encephalitis can progress rapidly to dee
coma.
This serious complication of varicella has a 5-20% mortality rate.
o Reye syndrome was associated with varicella when aspirin use was common. Identific
this association now has made acetaminophen the preferred drug, and Reye syndrome
become rare.
o Other neurological complications are aseptic meningitis, Guillain-Barré syndrome, and
polyradiculitis.
Pneumonia
o Pneumonia occurs primarily among older children and adults and can have a fatal outc
o Respiratory symptoms usually appear 3-4 days after the rash.
Herpes zoster
o A delayed complication of varicella, herpes zoster infection, occurs months to years aft
primary infection in about 15% of patients.
o The complication is caused by virus persisting in the sensory ganglions.
o Herpes zoster consists of a unilateral vesicular rash, limited to 1-3 dermatomes. The ra
often is painful in older children and adults. Among the health benefits of routine varice
immunization in childhood may be a lifelong decreased risk for reactivation of the virus
shingles.
Otitis media: About 5% of children with varicella develop otitis media, caused by the usual
pathogens.
Thrombocytopenia
Hepatitis is a self-limited accompaniment of varicella.
Glomerulonephritis
Hemorrhagic varicella
Prognosis:
Children with immunocompromised states are at risk for severe disease and death (eg, the m
rate among children with leukemia is 7%).
Patient Education:
Bathe the child regularly to reduce itching and prevent secondary infection.
o Wearing mittens or socks on the hands at night can help prevent scratching.
Advise parents to take children to the hospital if the following symptoms occur:
o Frequent vomiting
o Difficulty breathing, chest pain, wheezing, fast breathing, or severe cough
o Fever persisting more than 4 days or fever returns after defervescence
MISCELLANEOUS Section 9 of 11
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Medical/Legal Pitfalls:
Early recognition of secondary bacterial infection and appropriate follow-up are major issues.
to recognize occult infection may result in serious illness and even death.
Isolate patients with varicella because the disease is highly contagious and airborne spread c
occur. Isolation is especially important if the hospital also admits patients who are
immunocompromised because their exposure to the disease can be serious and even fatal.
Special Concerns:
Pregnancy is a particularly susceptible time. Varicella can cause various adverse outcomes fo
mother and infant, depending on the stage of pregnancy.
Immunocompromised children often have severe and complicated varicella, and their mortalit
is higher than that in immunocompetent children. Consider the following categories of patients
immunocompromised:
Skin diseases: Children with eczema or dermatitis may have severe skin manifestations durin
varicella.
PICTURES Section 10 of 11
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NOTE:
Medicine is a constantly changing science and not all therapies are clearly established. New research changes drug and treatment therapies daily. The authors,
and publisher of this journal have used their best efforts to provide information that is up-to-date and accurate and is generally accepted within medical standard
time of publication. However, as medical science is constantly changing and human error is always possible, the authors, editors, and publisher or any other
involved with the publication of this article do not warrant the information in this article is accurate or complete, nor are they responsible for omissions or errors i
article or for the results of using this information. The reader should confirm the information in this article from other sources prior to use. In particular, all drug do
indications, and contraindications should be confirmed in the package insert. FULL DISCLAIMER
Varicella excerpt