Hydroa Vacciniforme is an uncommon photodermatitis that often starts in childhood. Hydroa vacciniforme-like lymphoma (HVLL) is a lymphoproliferative disorder that can progress to systemic lymphoma.
Synonym: angiocentric cutaneous T-cell lymphoma of childhood
Hydroa vaccinforme has been associated with chronic Epstein-Barr virus (EBV) infection. HVLL is a NK or T cell lymphoproliferative disorder that is EBV positive.
Features of HVLL:
(1) The patient is usually a child or adolescent.
(2) The patient develops a photodermatitis with vesiculopapules in sun-exposed skin, consistent with hydroa vacciniforme.
(3) Biopsy of a skin lesion shows a lymphoproliferative disorder composed of monoclonal NK or T cells that are often angiocentric.
(4) The abnormal lymphocytes are positive for EBV by FISH.
(5) The condition often follows an indolent course.
(6) A patient may develop fever, lymphadenopathy or hepatosplenomegaly.
Complications:
(1) systemic lymphoma
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