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  Vol. 153 No. 8, August 1999 TABLE OF CONTENTS
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Pathological Case of the Month

Amir Bajoghli, MD; Franz E. Babl, MD, MPH; Robin L. Travers, MD
From the Departments of Dermatology (Drs Bajoghli and Travers) and Pediatrics (Dr Babl), Boston University School of Medicine, Boston Medical Center, Boston, Mass.

Arch Pediatr Adolesc Med. 1999;153:891-892.

A 15-MONTH-OLD African American boy with a history of atopic dermatitis since the age of 2 months was brought to the emergency department by his mother for an exacerbation of his chronic dermatitis. He had been regularly observed by his primary care physician and dermatologist, and the dermatitis was managed with topical corticosteroids and emollients. Over the 5 days prior to admission, he developed worsening pruritus, increased weeping lesions, irritability, and fever. He had no history of chickenpox; however, he had received a live, attenuated varicella vaccine (Varivax; Merck & Co, Inc, West Point, Pa) 5 days before admission. He had had contact with a visitor with "cold sores" 2 months earlier. On physical examination the child was irritable, uncomfortable, and constantly scratching. Rectal temperature was 40°C. Punched out erosions with an erythematous base were confluent on the face and more discrete on the trunk and upper extremities. Hemorrhagic and golden-colored crusting were evident and numerous excoriations were seen (Figure 1). Two erosions were noted on the soft palate mucosa, and the conjunctivae were normal. Shotty cervical, axillary, and inguinal lymphadenopathy was noted. A specimen for Tzanck testing was prepared by scraping the base of 1 of the facial erosions and staining the cellular material with Wright stain. Numerous multinucleated giant cells were noted on microscopic examination (Figure 2). Viral and bacterial skin cultures and bacterial blood cultures were obtained.


Figure 1.


Figure 2.


Diagnosis and Discussion

Eczema Herpeticum

Figure 1. Umbilicated and punched out papules and vesicles with yellowish and hemorrhagic crusts consistent with eczema herpeticum.

Figure 2. Tzanck test specimen of material scraped from the floor of a vesicle and stained with Wright stain revealing multinucleated giant cells (original magnification x40). Viral cultures revealed human herpesvirus 1.

Eczema herpeticum (EH), also known by its eponym Kaposi varicelliform eruption, is a potentially fatal, widespread cutaneous infection caused by human herpesvirus 1 (HHV-1). Eczema herpeticum is characterized clinically by clusters of umbilicated vesicles and eventually pustules in areas of previously abnormal skin. The most common predisposing cutaneous disorder is atopic dermatitis. Other less commonly reported risk factors for developing EH include Wiskott-Aldrich syndrome, Darier disease, pemphigus foliaceus, benign familial chronic pemphigus, and chronic irritant contact dermatitis.1 Cases that arise from healing second-degree burns,2 autografted skin,3 and staphylococcal scalded skin syndrome4 have been reported.

Abnormal skin is the virus' portal of entry, but the eruption rapidly spreads to previously normal and uninvolved skin. The vesicles evolve into pustules and then the classic punched out erosions, which may become confluent with subsequent hemorrhagic crusting. Patients are typically febrile and appear to have had a toxic reaction.5 Visceral dissemination of HHV-1 and subsequent mortality have been estimated at 1% to 9%.6 Secondary bacterial infection often complicates the course of illness and contributes to morbidity and mortality. The most common secondary pathogen is Staphylococcus aureus.5

The incidence of EH is highest in children younger than 3 years, with an equal male-female ratio.5 Approximately 3% of all children younger than 5 years have atopic dermatitis and are at risk of developing EH.7 Restriction fragment length polymorphism studies have shown that HHV-1 strains of the F35 genotype are more often associated with EH than other HHV-1 genotypes.8 Children with atopic dermatitis who have a number of circulating natural killer cells and decreased interleukin (IL)-2 and increased IL-4 levels are more susceptible to EH.9-10

Herein, we report the occurrence of EH 5 days after vaccination with a live, attenuated varicella vaccine. Initial evaluations raised concern whether the clinical findings were related to this. Results of the Tzanck test revealed multinucleated giant cells; however, this test cannot distinguish between HHV-1 or varicella-zoster infection. The HHV-1 infection was ultimately confirmed by conducting a viral culture. Bacterial cultures taken from the skin grew S aureus. Our patient promptly recovered after a parenteral injection of acyclovir and antistaphylococcal therapy (cephalexin oral suspension).

Neither review of all of the literature nor postmarketing surveillance by the manufacturer (Charlie A. Baechler, PhD, written communication, Merck & Co, Inc, July 25, 1997) has revealed any cases of EH after vaccination with the varicella vaccine. Adverse reactions to live, attenuated varicella vaccine include an injection site varicellalike rash with a median number of 2 lesions and a more generalized varicellalike rash with a median number of 5 lesions with the peak occurrence happening 5 to 26 days after vaccination.11

Pediatric patients with chronic cutaneous disorders are at increased risk of developing severe varicella infections, and active immunization with the varicella vaccine is not contraindicated.12 A high clinical suspicion for EH should be maintained in children with atopic dermatitis who have a vesicular eruption, even in the setting of recent varicella vaccination.


AUTHOR INFORMATION

Accepted for publication December 27, 1998.

Corresponding author: Amir Bajoghli, MD, Department of Dermatology and Pediatrics, Boston University School of Medicine, 609 Albany St, Boston, MA 02118.


REFERENCES

1. Flint ID, Spencer DM, Wilkin JK. Eczema herpeticum in association with familial benign chronic pemphigus. J Am Acad Dermatol. 1993;28:257-259. PUBMED
2. Bartralot R, Garcia-Patos V, Rodriguez-Cano L, Castells A. Kaposi's varicelliform eruption in a patient with healing second degree burns. Clin Exp Dermatol. 1996;21:127-130. PUBMED
3. Maners SM, Chetty BV. Eczema herpeticum occurring in auto-grafted skin. J Am Acad Dermatol. 1991;24:509-510. PUBMED
4. Barrio J, Lazaro P, Barrio JL. Kaposi's varicelliform eruption and staphlococcal scalded skin syndrome in adults. J Am Acad Dermatol. 1997;37:510-511. PUBMED
5. Novelli VM, Atherton DJ, Marshall WC. Eczema herpeticum. Clin Pediatr (Phila). 1988;28:231-233.
6. Atherton DJ, Marshall WC. Eczema herpeticum. Practitioner. 1982;226:971-973. WEB OF SCIENCE | PUBMED
7. Oakes RC, Cox AD, Walter HC, Burgdorf MD. Atopic dermatitis: a review of diagnosis, pathogenesis and management. Clin Pediatr (Phila). 1983;22:467-471.
8. Umene K, Yoshida M, Sakaoka H. Comparison of the association with eczema herpeticum in the two predominate genotypes of HSV-1. J Med Virol. 1996;49:329-332. PUBMED
9. Goodyear HM, McLeish P, Randall S, et al. Immunological studies of HSV infection in children with atopic eczema. Br J Dermatol. 1996;134:85-93. PUBMED
10. Raychaudhuri SP, Raychaudhuri SK. Revisit to Kaposi's varicelliform eruption: role of IL-4. Int J Dermatol. 1995;34:854-856. PUBMED
11. Varivax [package insert]. West Point, Pa: Merck & Co Inc; 1996.
12. American Academy of Pediatrics. 1997 Red Book: Report of the Committee on Infectious Diseases. 24th ed. Elk Grove Village, Ill: American Academy of Pediatrics; 1997:582.

SECTION EDITOR: ENID GILBERT-BARNESS, MD



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