Infectious Disease · PANCE / PANRE

Varicella (Chickenpox)

Primary VZV infection producing pruritic vesicular rash in successive crops; vaccine-preventable; reactivates years later as herpes zoster.

Also known as: chickenpox, VZV, varicella zoster virus, primary varicella

Overview

Primary infection with varicella-zoster virus (VZV, human herpesvirus 3) characterized by fever and a generalized pruritic vesicular eruption in successive crops. After resolution, VZV remains latent in sensory ganglia and can reactivate as herpes zoster.

Epidemiology

Universal vaccination (introduced in US in 1995) reduced incidence by >97%. Pre-vaccine: nearly all US adults were immune by age 30. Now seen primarily in unvaccinated children, vaccine breakthroughs (milder), and susceptible adults. Highly contagious by airborne and contact routes.

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Risk factors

  • Unimmunized status
  • Household and classroom contacts of an index case
  • Immunocompromise (severe disseminated disease, visceral involvement, death)
  • Pregnancy (severe maternal varicella pneumonia; congenital varicella syndrome 8-20 wk)
  • Neonatal exposure (mother varicella ±5 days of delivery)

Pathophysiology

Virus enters via respiratory tract, replicates in regional lymph nodes, undergoes primary viremia to liver/spleen, then secondary viremia seeds skin and mucosae. Infection of basal epidermal cells produces the characteristic 'dewdrop on a rose petal' vesicles. Latency is established in dorsal root and cranial nerve ganglia.

Clinical presentation

Symptoms

  • Prodrome (1-2 days, mild in children, severe in adults): fever, malaise, headache, anorexia
  • Rash begins on the scalp, face, and trunk and spreads peripherally
  • Lesions progress over hours: macule → papule → vesicle ('dewdrop on a rose petal') → pustule → crust
  • Successive crops mean lesions in all stages are present simultaneously — a key diagnostic feature
  • Intense pruritus

Signs / physical exam

  • 200-500 lesions in a typical case; fewer in vaccinated breakthroughs
  • Oral and genital mucosal vesicles
  • Patient is contagious from 2 days before rash until all lesions crust
  • Adults often have more lesions and higher fever

Classic findings

Lesions in multiple stages (papules, vesicles, crusts) simultaneously over the trunk and face.

Differential diagnosis

  • Smallpox (historical) — Uniformly aged lesions in a single stage, centrifugal distribution; vesicles deep-seated and umbilicated
  • Disseminated herpes zoster — Dermatomal vesicles with scattered satellite lesions in an immunocompromised host
  • Hand-foot-mouth disease (coxsackievirus) — Vesicles on hands, feet, oropharynx without generalized rash
  • Disseminated HSV — Grouped vesicles on an erythematous base, often mucocutaneous; PCR distinguishes
  • Bullous impetigo — Honey-colored crust, localized, S. aureus
  • Drug eruption — Generalized maculopapular rather than vesicular

Diagnostic workup

Diagnostic criteria

Clinical presentation of generalized vesicular rash with lesions in multiple stages; confirm with PCR or DFA if uncertain or in immunocompromised.

Labs

  • Diagnosis is usually clinical
  • VZV PCR of vesicular fluid — most sensitive when needed
  • Direct fluorescent antibody (DFA) of scraped vesicle base
  • Tzanck smear: multinucleated giant cells (does not distinguish VZV from HSV)
  • VZV IgG to document immunity

Imaging

  • Chest x-ray if pneumonia suspected (especially adults, smokers, immunocompromised)

Diagnostic algorithm

flowchart TD
  A[Exposure] --> B{Susceptible?}
  B -->|Immune| Z[No action]
  B -->|Yes - immunocompetent| C[Varicella vaccine<br/>within 3-5 d]
  B -->|Yes - high risk| D[VariZIG within 10 d<br/>± pre-emptive acyclovir]
  E[Clinical varicella] --> F{Host}
  F -->|Healthy child <13| G[Supportive<br/>no aspirin]
  F -->|Adolescent/adult| H[Oral acyclovir<br/>≤24 h of rash]
  F -->|Immunocompromised<br/>or severe| I[IV acyclovir]
Varicella exposure and disease management algorithm.

Treatment

First-line

  • Healthy children <13 years: supportive care — calamine, oral antihistamines, cool baths; keep nails trimmed to prevent superinfection; antipyretics with ACETAMINOPHEN (AVOID aspirin — Reye syndrome) and AVOID systemic NSAIDs given association with severe soft tissue infection
  • Adolescents ≥13 years and adults: oral acyclovir 800 mg 5x/day for 7 days (or valacyclovir 1 g TID) — start within 24 h of rash for best benefit
  • Immunocompromised, pregnant, or severe disease: IV acyclovir 10 mg/kg q8h
  • Varicella vaccine (live, 2-dose schedule) — primary prevention; first dose 12-15 months, second 4-6 years

Post-exposure prophylaxis

  • Susceptible immunocompetent contacts: varicella vaccine within 3-5 days of exposure
  • High-risk susceptible contacts (immunocompromised, pregnant, neonates exposed near delivery): VariZIG within 10 days of exposure
  • If VariZIG unavailable, IVIG or pre-emptive acyclovir starting day 7-10 post-exposure

Pregnancy

  • Avoid live varicella vaccine in pregnancy
  • Maternal varicella: oral acyclovir if within 24 h; IV acyclovir for pneumonia or severe disease
  • Maternal rash within 5 days before to 2 days after delivery → administer VariZIG to neonate plus IV acyclovir if symptomatic

Second-line / adjunct

  • Treat secondary bacterial skin infection (commonly group A strep or S. aureus) — beta-lactam coverage; consider necrotizing fasciitis with disproportionate pain
  • Airborne + contact precautions for hospitalized patients

Complications

  • Bacterial superinfection of skin lesions (group A strep, S. aureus, including necrotizing fasciitis and toxic shock)
  • Varicella pneumonia (most common cause of mortality in adults)
  • Cerebellar ataxia (most common neurologic complication in children; usually self-limited)
  • Encephalitis, transverse myelitis, vasculopathy
  • Reye syndrome (with aspirin use)
  • Congenital varicella syndrome (limb hypoplasia, cicatricial skin scars, microcephaly)
  • Neonatal varicella (severe if mother infected ±5 days of delivery)
  • Latency followed by herpes zoster decades later

PANCE pearls

  • Lesions in DIFFERENT stages simultaneously distinguish varicella from smallpox (uniform stages).
  • AVOID aspirin in children with varicella — Reye syndrome.
  • Disproportionate pain or violaceous skin in a varicella patient should raise concern for group A strep necrotizing fasciitis.
  • VariZIG within 10 days for high-risk exposed contacts — pregnant women, immunocompromised, neonates whose mothers were infected near delivery.
  • Two doses of varicella vaccine are recommended; breakthrough disease is milder and shorter.

References

  • ACIP 2007 — Prevention of Varicella: Recommendations of the ACIP (Marin et al., MMWR Recomm Rep 2007;56(RR-04))
  • AAP Red Book — American Academy of Pediatrics Red Book — Varicella-Zoster chapter
  • CDC — CDC Updated Recommendations for Use of VariZIG (MMWR 2013)

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