Clinical Features

Most affected patients are children or young adults of Asian (Japan, Korea, Taiwan) and Latin American origin (Mexico) (1-5). Edema, vesiculopapular eruptions, small blisters, crusts, necrotic areas, and scars occur mainly on the face and limbs (Figs. 1 and 2). The oral mucosae may be involved. The lesions are not induced or exacerbated by UV radiation. The history may reveal previous similar recurrent eruptions. There is often high fever, malaise, weight loss, failure to thrive, lymphadenopathy, and hepatosplenomegaly. Some patients report hypersensitivity to insect bites, which may be a trigger factor (6). Laboratory tests often show leukopenia, anemia, thrombocytopenia, and signs of impaired liver function. In addition, antibodies to EBV can be detected in the serum.

Figure 1 Vesiculopapules on the eyelid and cheeks. Note presence of conjunctivitis. (Courtesy of K. Iwatsuki. From Burg G, LeBoit Ph, eds. Unusual Lymphomas. Steinkopff-Verlag, 2001.)
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