{Reference Type}: Systematic Review
{Title}: Clinicoprognostic Study of Hydroa Vacciniforme-like Lymphoproliferative Diseases: A Systematic Review.
{Author}: Kim YJ;Yang HJ;Won CH;Chang SE;Lee MW;Lee WJ;
{Journal}: Dermatology
{Volume}: 239
{Issue}: 5
{Year}: 2023 May 25
{Factor}: 5.197
{DOI}: 10.1159/000530607
{Abstract}: <B>BACKGROUND: </B>Limited clinicopathological and prognostic data are available on hydroa vacciniforme (HV)-like lymphoproliferative diseases (HVLPD).<BR><B>METHODS: </B>This systematic review searched HVLPD reports in Medline via PubMed, Embase, Cochrane, and CINAHL databases in October 2020.<BR><B>RESULTS: </B>A total of 393 patients (65 classic HV, 328 severe HV/HV-like T-cell lymphoma [HVLL]) were analyzed. Among severe HV/HVLL cases, 56.0% were Asians, whereas 3.1% were Caucasians. Facial edema, hypersensitivity to mosquito bites, the onset of skin lesion, and percentage of severe HV/HVLL differed significantly by race. Progression to systemic lymphoma was confirmed in 9.4% of HVLPD patients. Death occurred in 39.7% patients with severe HV/HVLL. Facial edema was the only risk factor associated with progression and overall survival. Mortality risk was higher in Latin Americans than in Asians and Caucasians. CD4/CD8 double-negativity was significantly associated with the worst prognosis and increased mortality.<BR><B>CONCLUSIONS: </B>HVLPD is a heterogeneous entity with variable clinicopathological features associated with genetic predispositions.