EBV positive polyclonal or monoclonal T/NK LPD, characterized by blistering photodermatoses in childhood and healed with vacciniform scarring.

• Cutaneous form of CAEBV

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Clinical features

Classic type:

• Self-limited disease with vesicles on sun-exposed areas in adolescence/young adulthood

Severe HV-type:

• Extensive skin lesions
• Systemic manifestations: Fever, hepatomegaly, serologic abnormalities, and peripheral NK lymphocytosis
• Often progresses to EBV-associated NK/T-cell malignancy

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Diagnosis

Histopathology

• Epidermal reticular degeneration to spongiotic vesiculation with perivascular and periappendiceal lymphocytic infiltration with no definite cytologic atypia