Hydroa Vacciniforme-Like Cutaneous T-Cell Lymphoma

Abstract

Hydroa vacciniforme (HV)-like cutaneous T-cell lymphoma (HVLL) is a controversial skin pathology because some cases appear as hydroa vacciniforme, whereas others progress to cutaneous T-cell lymphoma with or without angiocentricity. It is usually associated with infections of Epstein Barr viruses and NK-cell lymphomas and typically affects the pediatric population. Symptoms include facial edema, papules, vesicles, and blisters in the facial region, arms, legs, and areas exposed to sunlight that leave varioliform scars. There may be infiltration of the lips, eyelids, and nose, usually accompanied by comorbid infections and hypersensitivity to insect bites. Frequency is rare, but HVLL more commonly affects patients from South America and Asia. Its clinical management can be difficult and accompanied by a high index of malignancy, thus early diagnosis is essential for effective and timely management.