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The clinicopathological features of 32 patients (17 females and 15 males) with a median age of 8 years (range, 1.5-21 years) from Southwestern China diagnosed with hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) were reviewed. At presentation, 6 patients showed only skin lesions, while 26 patients showed both skin lesions and systemic symptoms, including fever, lymphadenopathy and hepatosplenomegaly. As the disease progressed, systemic symptoms occurred in all patients. Follow-up data of 29 patients showed that 14 patients were still alive with disease with a median follow-up time of 22 months (range 3.6-71 months), and 15 patients died within a median follow-up of 6 months (range 0-60 months)., (© 2022 Wiley Periodicals LLC.)

Background: Hydroa vacciniforme (HV) is associated with Epstein-Barr virus (EBV) infection and a risk of transformation to lymphoma., Methods: We retrospectively analyzed six HV cases for EBV association and transformation to HV-like T-cell lymphoma. Clinicopathologic features were reviewed and cases were assessed for EBV-encoded RNA (EBER) by in situ hybridization, double staining with immunohistochemistry and EBER and for T-cell clonality., Results: The male-to-female ratio was 5:1, with a median age at diagnosis of 18.5 years. All patients initially had recurrent vesicles, necrotic ulcers or scars on sun-exposed areas. Symptoms were present before diagnosis between 2 weeks to 10 years. The mean follow-up time was 106.3 months. Four patients (67%) were EBV-positive. All four EBV-positive and one EBV-negative patients had relapsing clinical course. Double staining proved EBV infection in T-cells. Moreover, one EBV-positive patient developed HV-like T-cell lymphoma with hemophagocytosis after 209 months of recurrent papulovesicular eruptions and eventually died. T-cell clonality was successfully performed in four HV patients and all showed polyclonal results; the transformed HV-like T-cell lymphoma was monoclonal., Conclusions: In EBV endemic areas, HV is frequently (67%) associated with EBV infection, but transformation to HV-like T-cell lymphoma seems to be uncommon (17%) and bear a dismal outcome., (© 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

We report a case of hydroa vacciniforme-like skin eruptions with chronic active Epstein-Barr virus infection in a 16-year-old boy. The patient had repeated episodes of vesiculo-necrotic eruptions on the face, trunk, and extremities for 13 years. General symptoms included fever, splenomegaly, and abnormal liver function. Histopathological examination of skin biopsy specimen demonstrated dense infiltration of lymphocytes, histocytes, and some neutrophils and plasma cells in the dermis. Immunohistochemical stain revealed that the lymphocytes were positive for T cell and B cell markers. Epstein-Barr virus infection studies and systemic presentation confirmed the diagnosis., (© 2011 Wiley Periodicals, Inc.)

Hydroa vacciniforme-like cutaneous lymphoma is a rare type of Epstein-Barr virus-associated lymphoma. We analyze clinicopathologic features of seven Chinese child patients with hydroa vacciniforme-like cutaneous lymphoma and determine the pathogenic association of Epstein-Barr virus with this disorder. Clinical, histologic, and immunohistochemical features were reviewed. Skin lesions were subjected to Epstein-Barr virus-encoded RNA detection by in situ hybridization. Serologic assay and quantitation of Epstein-Barr virus DNA were performed. All seven patients presented with facial vesicles, papulovesicles, and atrophic scarring. Histologically, skin specimens showed epidermal blister formation, and dense lymphoid infiltration throughout the dermis and subcutaneous tissue. The infiltrate was composed of both small and large irregular lymphocytic cells expressing CD45RO or CD56. Tumor cells positive for Epstein-Barr virus-encoded RNA were detected in cutaneous infiltrates in all seven cases. Besides skin eruptions, all patients had systemic manifestations, such as intermittent fever, hepatosplenomegaly or lymph node enlargement. The amounts of Epstein-Barr virus DNA were increased in the peripheral blood in two detected cases, and antibody titers to Epstein-Barr virus revealed a chronic active Epstein-Barr virus infection in all cases. The diagnosis of hydroa vacciniforme-like cutaneous lymphoma was made. Four of seven patients were treated with interferon α intramuscularly, and the skin eruptions improved. Hydroa vacciniforme-like cutaneous lymphoma may present with severe facial edema, papulovesicular eruptions and permanent scars. The tumor cells often express natural killer- or T-cell markers. The disease is often preceded by chronic active Epstein-Barr virus infection., (© 2010 Wiley Periodicals, Inc.)

A 6-year-old boy had numerous episodes of hydroa vacciniforme. Several of these episodes were accompanied by an anterior uveitis with corneal clouding and stellate keratic precipitates. Wearing sunglasses prevented new eye lesions from developing despite recurrences of skin lesions. Phototesting on facial skin revealed reproduction of skin lesions with ultraviolet B but not ultraviolet A. One should be aware of eye involvement in hydroa vacciniforme, and children who experience this form of photodermatitis should have a careful eye examination and be advised to wear protective sunglasses.