Hydroa vacciniforme-like T-cell lymphoma: Report of two cases
DOI:
https://doi.org/10.18537/fouc.v04.n01.a06Keywords:
natural killer t-cells, hydroa vaccini- forme, epstein-barr virus infections, cutaneous t-cell lymphoma, lymphoproliferative disorders.Abstract
Introduction: Hydroa vacciniforme-like T-cell lymphoma is a rare condition characterized by recurrent papulovesicular, crusted, and ulcerated skin lesions, periorbital edema, lymphadenopathy, and, in some cases, progression to systemic lymphoma with a highly unfavorable prognosis. It typically occurs in individuals of indigenous descent in Latin American and Asian countries, with a higher incidence in children and young adults. In Ecuador, only a few cases have been documented despite the presence of several associated risk factors, such as ethnicity and sun exposure. This highlights the importance of reporting such cases, which may provide valuable insights for improving diagnosis and treatment strategies. Case presentation: Two male patients, aged 15 and 26, both of mestizo ethnicity and from rural areas, were evaluated. The first patient presented with ulcerative papuloedematous lesions on the face and upper limbs, along with erythematous-violaceous scaly plaques on the abdomen and lower limbs. The second patient exhibited a nodular edematous lesion and periorbital edema. Both were diagnosed with Epstein-Barr virus–positive hydroa vacciniforme-like T-cell lymphoma, although their prognoses differed. Conclusion: The lack of standardized protocols for the diagnosis and management of hydroa vacciniforme-like T-cell lymphoma remains one of the main challenges for timely detection and treatment. This limitation adversely affects patient prognosis and life expectancy, underscoring the urgent need to establish evidence-based clinical guidelines to improve therapeutic outcomes.
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