Eczema Herpeticum (Kaposi Varicelliform Eruption)
Eczema Herpeticum, Kaposi Varicelliform Eruption
Are You Confident of the Diagnosis?
What you should be alert for in the history
Sudden onset of a painful vesicular rash in a patient with a preexisting skin condition should alert you to the diagnosis of eczema herpeticum (EH).
Characteristic findings on physical examination
Characteristic findings on physical examination typically include vesicles and/or pustules that become hemorrhagic and crusted and eventually evolve into monomorphic punched out erosions (
Typical vesicles and punched out erosions on face and torso.
Typical vesicles and punched out erosions on hand and forearm.
Expected results of diagnostic studies
Diagnosis can be confirmed by direct fluorescent antibody (DFA), polymerase chain reaction (PCR), Tzanck smear, and viral culture. Biopsy is generally not necessary to make the diagnosis. Histopathologic changes are consistent with Herpes infection with ballooning degeneration of keratinocytes and multinucleated cells exhibiting viral cytopathic changes.
The differential diagnosis includes varicella, allergic contact dermatitis, bullous impetigo, eczema coxsackium, and eczema vaccinatum.
Who is at Risk for Developing this Disease?
EH most often occurs in patients with Atopic Dermatitis (AD) Patients with more severe AD, history of food allergies, history of asthma tend to be more susceptible to developing EH.
EH may also occur in the setting of any condition with disrupted epidermal barrier such as: contact dermatitis, seborrheic dermatitis, neurodermatitis, ichthyosis vulgaris, pemphigus foliaceus, Darier disease, Hailey-Hailey, pityriasis rubra pilaris, lupus erythematosus, pcsoriasis, Wiskott-Aldrich syndrome, congenital ichthyosiform erythroderma, mycosis fungoides, and Sézary syndrome. EH has also been reported in patients with recent dermabrasion, burns, skin graft, and laser treatments
What is the Cause of the Disease?
EH is caused by secondary viral infection of a preexisting dermatosis. It is typically caused by Herpes simplex virus (HSV) Type 1 (most common) or HSV Type 2.
Mutations in filaggrin have clearly been associated with atopic dermatitis and atopy. Recent studies have indicated that specific filaggrin mutations may confer increased suceptibility to EH.
Systemic Implications and Complications
Systemic implications/complications include viremia (especially in neonates and immunocompromised patients), secondary bacterial Infection/septicemia, and HSV keratitis. Because of the potential scarring and blindness, ophthalmology consultation is indicated with any suspicion of possible eye involvement.
Neonatal - intravenous route; 60mg/kg/day in three divided doses for a minimum of 14 days
Pediatric - intravenous route initially; May be transitioned to oral medication once stable
-15mg/kg/day in 3 divided doses for 5-7 days
-1200mg/day in 3 divided doses for 7-10 days; maximum 80mg/kg/day
Adults - Oral route; 400 mg orally 5x daily x 10 days
-Immunocompromised: intravenous route initially; May be transitioned to oral medication once stable
-Less than 12 years old: 30mg/kg/day in 3 divided doses for 7-14 days
-12 years old and older: 15mg/kg/day in 3 divided doses for 7-14 days
-Adults - 1000mg twice daily for 10 days
Foscarnet is the treatment of choice in immunocompromised adult patients with resistance to acyclovir -40 mg/kg IV Q8-12hr x 2-3weeks (maintain adequate hydration prior to and during treatment)
Note: Suppressive therapy is not generally necessary as recurrences are not common. Any recurrences of EH, however, should prompt consideration of prophylactic therapy
Optimal Therapeutic Approach for this Disease
If EH is suspected, early empiric treatment should be initiated immediately. For pediatric patients, initial intravenous therapy is the standard of care but may be transitioned to oral therapy once stable. Oral therapy may be considered in older pediatric patients with less severe and more localized disease. For Immunocompromised patients, initial intravenous therapy is the standard of care but may be transitioned to oral therapy once stable. If a patient is known to have acyclovir resistant herpes, Foscarnet is the treatment of choice
All patients require close follow-up to ensure adequate response to treatment. Patients not responding to typical outpatient medical therapy may require hospitalization for intravenous therapy
Unusual Clinical Scenarios to Consider in Patient Management
EH can be complicated by systemic viremia, especially in neonates/infants. Because of the increased morbidity and mortality of disseminated viral infection, management with hospitalization for systemic intravenous antivirals is the standard of care in the pediatric population. Older children may be transitioned oral medication once stable. Other possible complications that should not be missed include herpes keratitis and secondary bacterial infections and septicemia
What is the Evidence?
Wollenberg, A, Zoch, C, Wetzel, S, Plewig, G, Przybilla, B. "Predisposing factors and clinical features of eczema herpeticum: a retrospective analysis of 100 cases". J Am Acad Dermatol.. vol. 49. 2003. pp. 198-205.
Kramer, SC, Thomas, CJ, Tyler, WB, Elston, DM. "Kaposi's varicelliform eruption: a case report and review of the literature". Cutis.. vol. 73. 2004. pp. 115-22.
Wolverton, SE. Comprehensive Dermatologic Drug Therapy. Elsevier. 2007. pp. 102-124.
Pickering, LK, Baker, CJ, Kimberlin, DW. Red Book 2009 Report of the Committee of Infectious Diseases. American Academy of Pediatrics. 2009. pp. 777-778.
Beck, LA, Boguniewicz, M, Hata, T. "Phenotype of atopic dermatitis subjects with a prior history of eczema herpeticum". J Allergy Clin Immunol.. vol. 124. 2009. pp. 260-269.
Gao, P, Rafaels, NM, Hand, T. "Fillagrin mutations that confer risk of atopic deratitis confer greater risk of eczema herpeticum". J Allergy Clin Immunol. vol. 124. 2009. pp. 507-513.
Frisch, S, Siegfied, EC. "The clinical spectrum and therapeutic challenge of eczema herpeticum". J Pedi Dermatol. vol. 28. 2011. pp. 46-52.
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