Lymphoma & related disorders
Mature T/NK cell disorders
Pediatric NK/T cell disorders
Hydroa vacciniforme-like lymphoma


Topic Completed: 1 January 2012

Minor changes: 5 July 2020

Copyright: 2002-2020, PathologyOutlines.com, 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 lymphoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphomanonBhvl.html. Accessed October 26th, 2020.
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
Epidemiology
  • Mainly children and adolescents from Asia, or Native Americans from Central and South America, and Mexico
  • Rare in adults
Sites
  • Sun exposed skin, particularly the face (cheeks, nose, lower lip) but also ears and dorsum of hands
Etiology
  • 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
Treatment
  • 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 accompanied by an atypical lymphoid infiltrate and scattered neutrophils and eosinophils


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
  • EBER-ISH
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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