eMedicine Specialties > Dermatology > Viral Infections
Kaposi Varicelliform Eruption
Updated: Aug 29, 2007
Introduction
Background
Kaposi varicelliform eruption (KVE) is the name given to a distinct cutaneous eruption caused by herpes simplex virus (HSV) type 1, HSV-2, coxsackievirus A16, or vaccinia virus that infects a preexisting dermatosis. Most commonly, it is caused by a disseminated HSV infection in patients with atopic dermatitis.
Pathophysiology
To date, the pathophysiology of KVE remains unclear. Several proposed mechanisms involve both cell-mediated and humoral defects in persons with atopic dermatitis that could account for their susceptibility.
- T-cell–mediated immunity is important in the control of primary and recurrent HSV infections, and an HSV-specific, cell-mediated immune defect has been found in adults following KVE.
- Antibodies against HSV appear to limit the severity of infection and play a role in cell-mediated cytotoxicity. A persistently low enzyme-linked immunosorbent assay titer (1:1600) after infection has been reported following KVE.
- Natural killer (NK) cells provide a first-line defense against HSV infection, and reduced NK activity in atopic dermatitis has been reported. The reduced number of NK cells and a decrease in interleukin 2 receptors, a marker for lymphocyte activation, have been proposed to contribute to the susceptibility of children with atopic dermatitis.
Recent studies present conflicting data with regard to HSV-specific immune defects in patients with atopic dermatitis. One study failed to show any HSV-specific immune defect, either cell-mediated or humoral, in children with atopic dermatitis. In contrast, one study found that the skin in patients with atopic dermatitis is rich in interleukin 4 (IL-4) – producing CD4+ T cells. This increase in IL-4 can inhibit Th-1 cells and thereby suppress interferon-gamma secretion, leading to increased susceptibility to HSV infection in atopic skin.
The cathelicidin family of antimicrobial peptides is an integral component of the innate immune response that exhibits activity against bacterial, fungal, and viral pathogens. The importance of cathelicidins in antiviral skin host defense was confirmed by the observation of higher levels of HSV-2 replication in cathelicidin-deficient mouse skin compared with that seen in skin from their wild-type counterparts. Skin from patients with KVE exhibited significantly lower levels of cathelicidin protein expression than skin from patients with atopic dermatitis. An inverse correlation between cathelicidin expression and serum immunoglobulin E levels in patients with atopic dermatitis and patients with KVE has also been found. A high total serum immunoglobulin E level may also be a risk factor for the development of KVE.
Frequency
United States
The incidence of KVE has increased since 1980, likely secondary to the increased incidence of HSV infections.
Mortality/Morbidity
Significant morbidity and mortality can be associated with KVE due to HSV infection. However, with the introduction of intravenous acyclovir, in addition to systemic/topical antibiotic treatment, the mortality from KVE has decreased from as high as 50% to less than 10%.
Corticosteroid treatment has been suggested as a risk factor for developing KVE. However, a retrospective analysis of 100 cases of KVE showed that more than 75% of patients had not received corticosteroid treatment in the 4 weeks before the onset of KVE. This seems to argue against a role for topical steroids in the development of KVE. However, recent reports have described KVE occurring in atopic dermatitis patients treated with topical calcineurin inhibitors, such as tacrolimus, who subsequently developed KVE. Whether this is causally related remains unknown.
Sex
KVE affects men and women equally.
Age
- Originally thought to be a disorder of infants, it is now known to occur in children of any age and in adults.
- In a German study of 75 patients with KVE, the age of onset ranged from 5 months to 69 years. Most patients (56%) were aged 15-24 years.
- In one study, the mean age of onset of atopic dermatitis was lower (5.6 y) in patients with KVE compared with atopic dermatitis controls (9.6 y).
Clinical
History
- KVE begins as clusters of umbilicated vesiculopustules in areas where the skin has been affected by a preexistent dermatitis. Frequently, a delay in diagnosis occurs because the eruption is confused with the underlying disease until the characteristic umbilicated vesiculopustules appear. The eruption continues to spread over 7-10 days and may be associated with a high temperature, malaise, and lymphadenopathy.
- The primary episode of KVE runs its course and heals in 2-6 weeks.
- The average duration of illness is 16 days.
- Transmission occurs through contact with a person who is infected or by dissemination of primary or recurrent herpes.
- Recurrent episodes may also occur but are milder and not usually associated with systemic symptoms.
- Some studies have shown a high frequency of HSV DNA in the oral cavity of patients with KVE.
- In severe cases of KVE, lesions may heal with scarring.
Physical
- Umbilicated vesiculopustules that progress to punched-out erosions in the setting of a widespread dermatosis is virtually pathognomonic for KVE.
- The eruption is most commonly disseminated in the areas of dermatitis, with a predilection for the upper body and the head. Localized forms also exist.
- The vesicles often become hemorrhagic and crusted and can evolve into extremely painful erosions with a punched-out appearance.
- These erosions may coalesce to form large, denuded areas that frequently bleed and can become secondarily infected with bacteria.
Causes
- KVE is caused by HSV-1, HSV-2, coxsackievirus A16, or vaccinia virus infecting a preexisting dermatosis.
- Most commonly, it is caused by a disseminated HSV infection in patients with atopic dermatitis. For this reason, it is also referred to as eczema herpeticum.
- KVE has also been associated with mycosis fungoides, pityriasis rubra pilaris, neurodermatitis, irritant contact dermatitis, congenital ichthyosiform erythroderma, ichthyosis vulgaris, pemphigus foliaceus, benign familial pemphigus (Hailey-Hailey disease), Darier disease, Wiskott-Aldrich syndrome, Sézary syndrome, seborrheic dermatitis, skin grafts, burns, cowpox, and rosacea.
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References
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Further Reading
Keywords
eczema herpeticum, eczema vaccinatum, atopic dermatitis, herpes simplex virus type 1, HSV-1, herpes simplex virus type 2, HSV-2, coxsackievirus A16, vaccinia virus
