Lymphoma & related disorders
Mature T/NK cell disorders
Pediatric NK/T cell disorders
Hydroa vacciniforme-like LPD
Author: Dragos C. Luca, M.D.
Last author update: 1 January 2012
Last staff update: 27 December 2022
Copyright: 2002-2023, PathologyOutlines.com, Inc.
PubMed Search: Hydroa vacciniforme-like lymphoma
See also: Systemic EBV+ T cell Lymphoproliferative Disease of Childhood (S-EBV-TLPD)
Table of Contents
Definition / general | Epidemiology | Sites | Etiology | Postulated normal counterpart | Clinical features | Treatment | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Negative stains | Molecular / cytogenetics description | Molecular / cytogenetics images | Additional referencesCite this page: Luca D. Hydroa vacciniforme-like LPD. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomanonBhvl.html. Accessed June 9th, 2023.
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 America, 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
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
Additional references
