Hydroa vacciniforme-like lymphoproliferative disorder D47.

Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a very rare Epstein-Barr virus-associated lymphoproliferative and photosensitive disorder that occurs mainly in children in Latin America and Asia with a high vírus load. In Central Europe and North America, this variant has been observed only sporadically.

The initial clinical manifestations are mainly in sun-exposed areas (face, extensor extremities), but also in areas that have not been exposed to the sun, reddened facial oedema, vesicles and blisters, crusty ulcerations and small flat scars. The course is slowly progressive and recurrent. UV exposure may lead to recurrences.

The skin lesions are associated to a variable degree with systemic manifestations such as fever, weight loss, asthenia, arthralgia, lymphadenopathy, hepatosplenomegaly, and/or elevated liver enzymes.

In all cases EBV infections could be detected mostly with high viral load.

Atypical lymphocytic infiltrate in the dermis and/or subcutaneous tissue. The lesions show a cytotoxic T/NK cell immunophenotype. In a collective of 19 patients,12 cases showed a T cell phenotype. In these, molecular biology revealed 7x monoclonal rearrangements of T cell receptor genes. Three cases had an NK cell phenotype and showed polyclonal rearrangements in the TCR genes.

Varies according to the clinic. Indolent courses are treated symptomatically. In case of clinical indications of development of systemic lymphoma: chemotherapy.

Adequate light protection is important!

The majority of patients show an indolent, chronic course.

A smaller proportion of patients, especially those with atypical or severe skin lesions, may develop EBV-associated systemic T-cell or NK-cell lymphoma. Such clinical courses are observed mainly in children from Asia and Latin America with high EBV DNA levels, low numbers of NK cells and T cell clones in the blood. Cytopenia, elevated lactate dehydrogenase, destructive multiorgan involvement, and older age were poor prognostic factors.

1. Chen CC et al. (2020) Hydroa Vacciniforme and Hydroa Vacciniforme-Like Lymphoproliferative Disorder: A Spectrum of Disease Phenotypes Associated with Ultraviolet Irradiation and Chronic Epstein-Barr Virus Infection. Int J Mol Sci 21:9314.
2. Guo N et al. (2019) Clinicopathological categorization of hydroa vacciniforme-like lymphoproliferative disorder: an analysis of prognostic implications and treatment based on 19 cases. Diagn Pathol 14:82.
3. Iwatsuki Ket al. (2019) Hydroa vacciniforme: a distinctive form of Epstein-Barr virus-associated T-cell lymphoproliferative disorders. Eur J Dermatol 29: 21-28.
4. Han B, Hur K, Ohn J, Kim TM, Jeon YK, Kim YC, Mun JH. Hydroa vacciniforme-like lymphoproliferative disorder in Korea. Sci Rep 2020 Nov 9;10(1):19294.

5. Wang Mi (2013) Hydroa vacciniforme-like lymphoma of an adult: a case report with review of the literature. Diagn Pathol 8: 72.