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Internal Medicine

Hydroa vacciniforme (HV)-like LPD

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

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