8-year-old girl presents to ED with painful rash and fever
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An 8-year-old female presented to the ED with a painful eruption for 6 days’ duration. She had a history of moderate eczema that was flaring for the past few weeks and was being managed with topical steroids.
She denied any history of cold sores. Six days before presentation, she developed bumps and sores on her arms and legs that quickly multiplied. She also developed fevers and initially presented to an urgent care center, where she was given topical bacitracin. Her skin continued to worsen, and her fevers persisted. She subsequently presented to the ED for evaluation.
In the ED, she was noted to be febrile to 102°F with rigors. Physical exam revealed numerous monomorphic papules, pustules and round erosions, many of which coalesced into larger eroded plaques with scalloped borders (Figure 1). The arms, legs and lower trunk were the most prominent areas affected (Figure 2).
Can you Spot the Rash?
A. Atopic dermatitis flare
B. Eczema herpeticum
C. Varicella
D. Eczema coxsackium
Case Discussion
Kaposi varicelliform eruption, also known as eczema herpeticum (EH) — correct choice, B — is a severe cutaneous eruption due to atopic dermatitis (AD) with herpes simplex virus (HSV) superinfection. EH can occur in other disease states that also cause impaired skin barrier function, including Darier disease and autoimmune blistering diseases. EH is rare despite the high prevalence of AD.
EH is characterized by an abrupt onset of fever with a skin rash composed of painful, monomorphic papules, pustules, vesicles and “punched-out” appearing erosions. When the lesions group together, they can form larger, eroded areas, often with scalloped borders. The lesions are typically localized to areas of the body that are most affected by underlying dermatitis. These patients are often ill-appearing with systemic symptoms and can require admission to the hospital for management if widespread or the near the eyes.
Given that AD is a condition characterized by cutaneous immune dysregulation and impaired barrier function, children with eczema are at risk for superimposed bacterial and viral skin infections. The differential diagnosis of a vesicular and erosive rash in a child with eczema should therefore always include infectious etiologies such as HSV, varicella, enterovirus, staphylococcus and streptococcus. Clinical clues that point toward EH include abrupt onset of fever with characteristic monomorphic and “punched-out” appearing lesions. A swab of the blister fluid for HSV PCR will confirm the diagnosis. When the characteristic lesions are not present, or the diagnosis is unclear, viral PCRs for varicella and enterovirus should be obtained as well. A bacterial culture from lesional fluid is also important to collect because EH can be complicated by bacterial superinfection. This patient was superinfected with methicillin-sensitive Staphylococcus aureus as well as Streptococcus dysgalactiae. Patients with EH are often systemically unwell; standard sepsis workup including routine labs and blood cultures should also be performed when indicated. Viremia is another complication of EH, and this patient had HSV-positive PCRs in both the skin and blood.
To treat EH, early administration of antiretroviral therapy with acyclovir is key and is often given intravenously. Early initiation with acyclovir has been shown to improve outcomes in hospitalized children. Therefore, if the clinical suspicion for EH is high, one should not delay starting therapy while waiting for testing to result. Antibacterial therapy to cover for both staphylococcus and streptococcus should also be considered if there is a concern for bacterial superinfection. IV fluid resuscitation may be needed to correct for fluid losses. Skin care to help repair the barrier should involve the use of bland emollients such as plain petrolatum, as well as topical anti-inflammatory agents to treat the underlying dermatitis. For patients with facial involvement, an ophthalmology consult is warranted to evaluate for keratoconjunctivitis. Prophylactic acyclovir may be needed in patients who experience recurrence. Overall, the mortality from EH is low.
References:
- Aronson PL, et al. Pediatrics. 2011;doi:10.1542/peds.2011-0948.
- Eichenfield LF, et al. Neonatal and Infant Dermatology. 3rd ed. London: Elsevier; 2015.
For more information:
- Michele Khurana, MD, is a pediatric dermatology fellow at The Children’s Hospital of Philadelphia. She can be reached at khuranam@email.chop.edu.
- Marissa J. Perman, MD, is an attending physician at The Children’s Hospital of Philadelphia.
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