Lymphoma & related disorders

Mature T/NK cell disorders

Pediatric NK/T cell disorders

Hydroa vacciniforme-like lymphoproliferative disease

Last author update: 1 January 2012
Last staff update: 24 November 2023

Copyright: 2002-2024,, Inc.

PubMed Search: Hydroa vacciniforme-like lymphoma

See also: Systemic EBV+ T cell Lymphoproliferative Disease of Childhood (S-EBV-TLPD)

Dragos C. Luca, M.D.
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Cite this page: Luca D. Hydroa vacciniforme-like lymphoproliferative disease. website. Accessed February 23rd, 2024.
Definition / general
  • Chronic EBV+ cutaneous lymphoproliferative disorder with a broad clinical spectrum, a usually protracted clinical course and a long term risk to progress to a systemic lymphoma
  • Occurs in children and associated with sun sensitivity (WHO 2008)
  • Increased frequency in Asians and Native Americans from Central and South America, and Mexico
  • Mainly children and adolescents from Asia or Native Americans from Central America, South America and Mexico
  • Rare in adults
  • Sun exposed skin, particularly the face (cheeks, nose, lower lip) but also ears and dorsum of hands
  • EBV transformed neoplastic cells (usually T but sometimes NK cells)
  • Hypersensitivity to sunlight
  • Related condition: mosquito bite hypersensitivity (the EBV+ cells are NK cells)
Postulated normal counterpart
  • Skin homing cytotoxic T cell or NK cell
Clinical features
  • Papulovesicular eruption usually followed by ulceration and scarring (mimics herpes)
  • Edema of face, eyelids and lips
  • Systemic symptoms (fever, wasting, lymphadenopathy, hepatosplenomegaly, myocarditis) may occur, particularly late in course of disease
  • Variable clinical course with recurrent skin infections for up to 10 - 15 years before progression to systemic involvement
  • Much more aggressive once systemic spread has occurred
  • Mosquito bite allergy: clinically more aggressive and often associated with a hemophagocytic syndrome
Microscopic (histologic) description
  • Small to medium neoplastic cells without significant atypia
  • Infiltrates extend from epidermis to subcutis with necrosis, angiocentricity, angioinvasion, epidermal ulceration
Microscopic (histologic) images

Contributed by Hillary Rose Elwood, M.D.

Shave biopsy shows epidermal spongiosis, vesiculation and necrosis

Lymphocytic infiltrate is CD3+, TIA+, subset weak CD4+, subset CD30+ and negative for CD8 and CD56

Positive stains
  • Cytotoxic T cell phenotype; less often NK cell phenotype with CD56 expression
Negative stains
Molecular / cytogenetics description
  • Clonal TCR gene rearrangements in most cases (except some NK cell cases)
  • EBV is monoclonal
Molecular / cytogenetics images

Contributed by Hillary Rose Elwood, M.D.

EBER in situ hybridization

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