Pediatric II – Module IVDr. Christine Bernal

B4

Case 4: “Ang Pamana”

Guide Questions1. What conditions present with vesiculopustular

eruptions?2. What kind of Varicella infection does this infant have?3. Differentiate the following: Neonatal Varicella,

Congenital Varicella Syndrome and Herpes Zoster?4. What is the treatment for Varicella?5. What are the complications of Varicella?6. Identify the individuals who would need treatment with

either Acyclovir or VZIG?7. How would you prevent Varicella (Primary prevention

and Postexposure prophylaxis)?

What conditions present with vesiculopustular eruptions?

Infectious• Viral– Varicella-zoster virus– Hespes simplex virus 1 and 2– Coxsackievirus A4, A5, A7-10 A16 and B1-3, B5– Echonovirus 4, 6, 9, 11, 17, 19, 33– Enterovirus 7,2

• Bacterial– S. aureus– S. pyogenes

Non Infectious

• Drug reaction• Stevens-Johnson syndrome• Anthropod bites• Contact dermatitis• Erythema Multiforme• Thermal Injury• Toxic epidermal necrolysis

What kind of Varicella infection does this infant have?

Clinical Diagnosis of Varicella Diagnostic features:

Papulovesicular eruptions associated with fever and mild constitutional symptoms

Rapid progression of macules to papules to vesicles and finally to crusts

Appearance of symptoms simultaneously in one anatomic area

Predominant central distribution of lesions including the scalp

Eventual crusting of all skin lesions

Varicella-zoster infection

• Skin lesions are first seen on the body and inner aspects of the thighs but spread quickly to the face, scalp and proximal parts of the limbs

Neonatal Varicella• Infants whose mothers develop

varicella in the period from 5 days prior to delivery to 2 days afterward

• Infant acquires the infection transplacentally as a result of maternal viremia, which may occur up to 48 hr prior to the maternal rash

• Infant's rash may occur toward the end of the 1st week to the early part of the 2nd week of life

VZD Diagnosis

• Diagnosis is usually made clinically– The presence of a herpesvirus can be

demonstrated by a Tzanck smear that demonstrates inclusions

– Infection can be confirmed by acute and convalescent titers of VZV antibody

Differentiate the following:Neonatal Varicella

Congenital Varicella Syndrome Herpes Zoster

Neonatal Varicella• Infants whose mothers develop varicella in the

period from 5 days prior to delivery to 2 days afterward.

• High mortality• Transplacental, which may occur 48 hrs. prior to

the maternal rash.• Infant rashes may occur during 1st wk or early 2nd

wk of life• Since the mother does not develop the antibody,

the infant receives a large dose of virus without maternal anti –VZV antibody

Neonatal Varicella

• If the mother was more than infected 5 days, she can still pass the virus to her child, but in a milder form due to her anti-VZV.

• Treatment:– 1 vial of human varicella-zoster immune globulin

(VariZIG)– Acyclovir (10mg/kg q8hrs IV) when lesions

develop

Congenital Varicella

• Infants whose mothers develop varicella early in pregnancy.– Gestational period. Major development and

innervation of the limb buds and maturation of the eyes

Time of infection Organ system involved

6-12wk of gestation Maximal interruption of Limb development

16-20 wk of gestation Eye and Brain development

Congenital VaricellaStigmata of Varicella-Zoster Infection

Damage to Sensory Nerves Cicatricial skin lesionsHypopigmentation

Damage to Optic Stalk and Lens Vesicle MicrophthalmiaCataractsChorioretinitisOptic Atropy

Damage to Brain/encephalitis MicrocephalyHydrocephalyCalcificationsAplasia of the Brain

Damage to the Cervical or Lumbosacral cord

Hypolasia of an extremityMotor and sensory deficitsAbsent DTRHormer syndromeAnal/urinary sphincter dysfuction

Congenital Varicella

• Diagnosis– Maternal history– PCR– Fetal cord sampling and Chorionic villus sampling

(detection of viral DNA, virus or antibody)

Persistent positive VZV IgG antibody titer after 12-18months of age is indicator of prenatal infection in an asymptomatic child.

Congenital Varicella

• Prevention– Vaccination of the mother of the varicella Vaccine

3 months prior to pregnancy

Herpes Zoster

• Vesicular lesions clustered within 1 or less commonly 2 adjacent dermatomes

Elderly ChildrenBurning painClusters of skin lesions in a dermatomal patternPost herpetic neuralgia (complication)

Mild rashInfrequently assoc. with localized pain, hyperesthesia, pruritus and low grade feverSymptoms of acute Neuritis are minimalComplete resolution within 1-2 wks

Herpes Zoster

Herpes Zoster

• Inc. risk – Acquired varicella infection in the 1st yr of life– Mothers have varicella infection in the 3rd

trimester of pregnancy– Immunocompromised • Can have disseminated cutaneous disease that mimics

varicella, visceral dissemination with pnueumonia, hepatitis, encephalitis and DIC

Herpes Zoster

• treatment

Healthy Adult Acyclovir (800mg 5x a day PO for 5 days)Famciclovir (500mg tid PO for 7 days)Valacyclovir(1000mg tid PO for 7 days)

Healthy children Supportive therapyOr with oral acyclovir (20mg/kg/dose, max 800 mg/dose)

Immunocompromised children

Acyclovir (500mg/m2 or 10mg/kg q8hr IV)

What is the treatment for Varicella?

Treatment

• 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.

Acyclovir

• Antiviral that acts by inhibiting herpes virus DNA polymerase and terminating viral replication.

• It reduces the number of lesions and duration of fever if started within 24 h of appearance of rash.

• Available as cap (200-800 mg), PO liquid (400 mg/5 mL), and parenteral injection (500 mg/mL

• Pediatric• 80 mg/kg/d PO divided in 4-5 doses for 5 d;

not to exceed 3200 mg/d

• 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

What are the complications of Varicella?

Complications• Bacterial Infections– usually caused by group A Streptococci (the most common)

and S. aureus– range from superficial impetigo to cellulitis, lymphadenitis,

and subcutaneous abscesses– Erythema of the base of a new vesicle

• early manifestation of secondary bacterial infection – Recrudescence of fever

• 3–4 days after the initial exanthem

– Varicella vaccine

Complications Pneumonia

The frequency of varicella pneumonia may be greater in the parturient and may lead to premature termination of pregnancy.

Recognized chiefly in otherwise healthy adults and immunocompromised children

Severe complication Respiratory symptoms○ cough, dyspnea, cyanosis, pleuritic chest pain, and hemoptysis,

usually begin within 1–6 days after the onset of the rashSmoking ○ a risk factor for severe pneumonia complicating varicella

Complications• Encephalitis and Cerebellar Ataxia– patients younger than 5 yr or older than 20 yr. – high

morbidity – Neurologic symptoms:

• nuchal rigidity, altered consciousness, and seizures • usually begin 2–6 days after the onset of the rash but may occur

during the incubation period or after resolution of the rash

– Cerebellar ataxia have a gradual onset of gait disturbance, nystagmus, and slurred speech

– Clinical recovery: • rapid, occurring within 24–72 hr, and is usually complete

– Reye syndrome of encephalopathy and hepatic dysfunction associated with varicella • rare; salicylates are no longer routinely used as antipyretics

Complications

• Mild thrombocytopenia – occurs in 1–2% of children – may be associated with transient petechiae

• Rare complications – Purpura, hemorrhagic vesicles, hematuria, and

gastrointestinal bleeding are that may have serious consequences.

6. Identify the individuals who would need treatment with

either Acyclovir of VZIG.

Acyclovir• Children with defects in cell-mediated immunity,

chronic atopic dermatitis or asthma, iatrogenic immunosuppression or long-term systemic steroid use, splenic dysfunction, nephrotic syndrome high risk for varicella-related complications

• Healthy adults at increased risk of severe varicella infections

• Immunocompromised/immunosuppressed populations

VZIG• Highly susceptible, VZV-exposed

immunocompromised or immunosuppressed populations– Bone marrow transplantation– Leukemia– Congenital or acquired immunodeficiency syndromes– Undergoing immunosuppressive therapy for

transplant procedures– Infants born to mothers who experience onset of

chickenpox five days prior to delivery or within two days after delivery

7. How would you prevent Varicella ( Primary prevention

and Postexposure prophylaxis)?

Primary Prevention - Varicella vaccine

• susceptible children aged 12 months to 12 years• Live-attenuated preparation of serially propagated and

attenuated wild Oka strain• Dose: 0.5 ml subcutaneously(recommended) or IM, one dose

for children <12 yrs or younger and 2 doses 4-8 weeks apart for individuals older than 12 yrs of age– 95% immunogenic for immunized healthy children

between 12 mos and 12 yrs of age with humoral and CMI response

– 78-82% after 1 dose and 99% after 2 doses for people 13 yrs or older

– Duration of immunity: at least 11 yrs (USA); 20 yrs (Japan studies)

Primary Prevention - VZIG• High Risk:– Immunocompromised, susceptible children without

history of varicella or varicella immunzation– Normal susceptible adults, especially pregnant

women– Newborn infant of a mother who had onset of

Chickenpox within 5 days before or 48 hours after delivery

– Hospitalized premature infant (>28 wks gestation) whose mother has not had chickenpox

– All hospitalized premature infants <28 wks gestation or weighing <1,000 gms regardless of maternal historyof varicella

Post-exposure Prophylaxis• IV Acyclovir should be given to

immunocompromised patients with Varicella or Herpes zoster

• Oral acyclovir given to healthy children with varicella within 24 hrs of the rash results in diminution and duration of skin lesions. Should be considered in adolescents and adults with Varicella

• VZIG should be given within 96 hrs of exposure to susceptible high risk patients for severe or complicated Varicella

Thank you