Contents
EBV positive polyclonal or monoclonal T/NK LPD, characterized by blistering photodermatoses in childhood and healed with vacciniform scarring.
- Cutaneous form of CAEBV
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
Diagnosis
Histopathology
- Epidermal reticular degeneration to spongiotic vesiculation with perivascular and periappendiceal lymphocytic infiltration with no definite cytologic atypia