Hydroa vacciniforme‐like lymphoproliferative disorder: A retrospective study on clinicopathological characteristics of 32 cases

Abstract

Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is an Epstein-Barr virus (EBV)-associated cutaneous T/NK cell lymphoproliferative disease that mainly affects children and adolescents. 1 Classic hydroa vacciniforme (HV) is characterized by papulovesicular eruptions that evolve into crusts and heal with pitted scars in sunexposed areas. Systemic symptoms, including fever, lymphadenopathy, and hepatosplenomegaly, are absent. Atypical or severe HV has mainly been reported in East Asia and Latin America. These patients present with more progressive skin eruptions, such as marked facial edema, necrosis, or large ulcers, which usually extend to non-sunexposed areas and are accompanied by systemic symptoms. 2 2 | B RIEF REP ORT This retrospective study was performed at the Affiliated Children's Hospital and the First Affiliated Hospital of Chongqing Medical University.Thirty-two patients evaluated from 2013 to 2020 were included (Table 1). Patients included 17 females and 15 males, ranging from 1.5 to 21 years (median: 8 years) at time of diagnosis.Thirty-four biopsies of skin lesions from 32 patients were analyzed. Epidermal vesicles, ulcers, and necrosis were observed, with varying degrees of small-to medium-sized polymorphic lymphocytes infiltrating the dermis and subcutaneous fat, with a predominantly perivascular and periadnexal distribution.

Photodermatoses: what's new

“…Another retrospective study of 32 patients between 1.5–21 years of age found that at presentation, 6 patients only had skin lesions, whereas 26 patients showed both skin lesions and systemic symptoms, including fever, lymphadenopathy, and hepatosplenomegaly. As the disease progressed, systemic symptoms occurred in all patients, and after a median follow-up time of 6 months, 15 patients had died [6 ▪ ]. Finally, a study of nine patients found that eight patients who received combinations of thalidomide, interferon, prednisone, acyclovir, and/or isotretinoin improved, whereas one patient who received chemotherapy died, corroborating reports noting that traditional chemotherapy may be associated with poor outcome [7].…”

Hydroa Vacciniforme Lymphoproliferative Disorder in a Young Spanish Woman: An Infrequent Case With Fatal Outcome

“…Through the report of a series of patients, some groups have provided new information that has allowed us to recognize a broader spectrum of the disease, both clinically and with some variability in the histopathological features as well. [4][5][6][7][8] Here, we present an original case of an HV LPD with an atypical clinical presentation and a fatal outcome.…”

“…Through the report of a series of patients, some groups have provided new information that has allowed us to recognize a broader spectrum of the disease, both clinically and with some variability in the histopathological features as well. 4–8…”