Lymphoma and plasma cell neoplasms
T / NK cell disorders
Hydroa Vacciniforme-like Lymphoma (HVL)

Author: Dragos C. Luca, M.D.

Revised: 3 July 2018, last major update January 2012

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

PubMed Search: Hydroa Vacciniforme-like Lymphoma

See also: Systemic EBV+ T cell Lymphoproliferative Disease of Childhood (S-EBV-TLPD)
Cite this page: Luca, D. Hydroa Vacciniforme-like Lymphoma (HVL). PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/lymphomanonBhvl.html. Accessed July 16th, 2018.
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

Images hosted on PathOut server:

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

Images hosted on PathOut server:

Contributed by Hillary Rose Elwood, M.D.

EBER in situ hybridization