Ocular Eczema Herpeticum

Photoclinic

A 9½-year-old boy was referred to our ophthalmology service for evaluation of a 3-day history of bilateral eyelid rash. At presentation, he had been hospitalized with a 5-day history of a high fever of 40.6°C (105°F). His past medical history was significant for gastroesophageal reflux disease, eczema, accommodative esotropia, and herpetic stomatitis. His medications were nasal fluticasone, a topical corticosteroid cream for eczema, and diphenhydramine.

General examination showed a bilateral upper and lower eyelid rash characterized by pustules located on an erythematous base. There was some crusting of the lesions but no purulent discharge. A similar rash was present on the vermillion border, the left side of the neck, and the surface of the penis. Other than the eyelid rash, ocular examination findings were unremarkable.

Potassium hydroxide staining results were negative for fungi. Viral culture was positive for herpes simplex virus type 1 (HSV-1). The patient received a diagnosis of eczema herpeticum (EH) and was started on a regimen of 400 mg of oral acyclovir 3 times a day. One week after initiation of therapy, all lesions had resolved completely, and the patient was afebrile.

EH, also called Kaposi varicelliform eruption, is a cutaneous eruption of viral etiology that arises in a preexisting dermatosis. It presents as clusters of umbilicated vesiculopustules, most commonly on the upper body and head, and it may be associated with fever, malaise, lymphadenopathy, and lymphocytopenia.¹ The vesicles may spread and become hemorrhagic and crusted, leading to painful, punched-out erosions that often coalesce into large, bleeding erosions.² The primary episode of EH usually lasts between 2 and 6 weeks, with an average of 16 days. Recurrent episodes are possible but generally are milder and are not frequently associated with systemic symptoms.

The most common cause is HSV-1 or -2, although EH also can result from coxsackievirus A16 or vaccinia virus infections. EH occurs most commonly in children and young adults, although its true incidence is unknown owing to its infrequency and a lack of large-scale studies.³

While the pathophysiology of EH is unclear, the essential predisposing factor is disruption of the stratum corneum. EH most commonly occurs secondary to atopic dermatitis (AD), and it is more likely to develop in persons with more severe AD,^1,4 a history of asthma and/or food allergy,⁴ an earlier onset of AD,¹ or significantly higher serum immunoglobulin E levels.¹

The most useful and reliable diagnostic laboratory tests are viral cultures and direct fluorescent antibody (DFA) staining of cells scraped from lesions. Both methods can identify HSV-1, but DFA staining can yield results within a few hours, compared with at least 48 hours for viral cultures.

An ophthalmologist should be consulted when ocular involvement is present or suspected. Ocular herpetic infection is rare, but herpetic keratitis is possible and can lead to scarring. Other complications of EH include herpes hepatitis, disseminated intravascular coagulation,² and bacterial superinfection, most commonly with Staphylococcus species.⁵

Before the advent of antiviral therapies, fatalities from EH were common.² Because of the severe and potentially life-threatening complications of EH, treatment should be initiated as soon as there is a high suspicion of disease. Acyclovir is the most common initial therapy; other antiviral therapies include valacyclovir and vidarabine, as well as foscarnet for immunocompromised patients and patients with acyclovir-resistant HSV. Patients with recurrent HSV infection and a chronic skin disease that predisposes them to EH can be treated prophylactically with oral acyclovir or oral valacyclovir. If no evidence of bacterial superinfection is present, an antibiotic cream such as silver sulfadiazine can be used prophylactically.

References:

1. 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. 2003;49(2):198-205.

2. Mackley CL, Adams DR, Anderson B, Miller JJ. Eczema herpeticum: a dermatologic emergency. Dermatol Nurs. 2002;14(5):307-310,323.

3. Olson J, Robles DT, Kirby P, Colven R. Kaposi varicelliform eruption (eczema herpeticum). Dermatol Online J. 2008;14(2):18.

4. Beck LA, Boguniewicz M, Hata T, et al. Phenotype of atopic dermatitis subjects with a history of eczema herpeticum. J Allergy Clin Immunol. 2009;124(2):260-269.

5. Brook I, Frazier EH, Yeager JK. Microbiology of infected eczema herpeticum. J Am Acad Dermatol. 1998;38(4):627-629.