Clinical Dermatology Review

: 2019  |  Volume : 3  |  Issue : 2  |  Page : 139--141

Hydroa vacciniforme-like lymphoma

Zhang Fang, Yang Huizhi, Shi Jianqiang, Lin Jiaxi, Cai Yanxia, Chen Rongyi 
 Department of Dermatology, Affiliated Hospital of Guangdong Medical University, Zhanjiang, China

Correspondence Address:
Chen Rongyi
Department of Dermatology, Affiliated Hospital of Guangdong Medical University, Zhanjiang 524001


Hydroa vacciniforme-like lymphoma is a rare cutaneous T-cell lymphoma occurring in children associated with Epstein–Barr virus (EBV) infection. We reported a 15-year-old Chinese boy who presented with a 3-month history of recurrent special clinical manifestations including vesicles, erythematous papules, ulcer, crusting, and smallpox-like scar on his head, face, neck, limbs, and ankles. EBV-DNA level was positive. Immunohistochemistry showed CD2(+++), CD3(+++), CD4(+), CD5(++), CD7(+), CD8(±), CD56(+), ki67 (30%+), Gr-B(+++). The patient showed a significant improvement in clinical symptoms after being treated with interferon alpha and prednisone.

How to cite this article:
Fang Z, Huizhi Y, Jianqiang S, Jiaxi L, Yanxia C, Rongyi C. Hydroa vacciniforme-like lymphoma.Clin Dermatol Rev 2019;3:139-141

How to cite this URL:
Fang Z, Huizhi Y, Jianqiang S, Jiaxi L, Yanxia C, Rongyi C. Hydroa vacciniforme-like lymphoma. Clin Dermatol Rev [serial online] 2019 [cited 2020 Feb 23 ];3:139-141
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Full Text


Hydroa vacciniforme (HV)-like lymphoma is a cutaneous cytotoxic T-cell or natural killer cell-originated lymphoma mainly occurring in children.[1],[2] Many cases were reported from Asia and South America. In the updated of 2008 WHO classification of lymphoid neoplasms, hydroa vacciniforme-like lymphoma has been recognized as one of the Epstein–Barr virus (EBV)-positive lymphoproliferative disorders of childhood.[3],[4] We reported an exceptional case of EBV-associated T/natural killer (NK) cell lymphoproliferative disorder in a boy who shares similar characteristics of HV.

 Case History

A 15-year-old boy, and reports in this study had been approved by the patient's parents. Presented to our department with a 3-month history of recurrent skin lesions, accompanied with fever. Three months ago, the skin lesions showed a slowly progressive relapsing course, changing from papules, vesicles, necrosis, and finally healing with atrophic scars. On physical examination, variously sized edemas, erythematous papules, vesicles, and crusts were along with some pitting scars on his face, neck, arms, and legs [Figure 1]. His legs and ankles became edema 20 days ago. During these days, he had high fever and emesis. When he was an inpatient in our hospital, clinically, HV-like lymphoma was suspected. Laboratory examinations showed that red blood cell was 4.57 × 1012/L, white blood cell was 3.1 × 109/L, blood platelet was 72.0 × 109/L, lymphocyte T was 446/u, erythrocyte sedimentation rate was 31 mm/h, aspartate aminotransferase was 63.3 U/L, ALP, alkaline phosphatase was 119.7 U/L, lactate dehydrogenase was 560.5 U/L, and immunoglobulin E was 1044.01 U/L. Ultrasonic examination revealed slight enlargement of the liver and spleen. Moreover, his cervical lymph node enlargement too, the bigger one was 2.3 cm × 0.6 cm (left lymph node). EBV-DNA level was positive, detected by real-time quantitative polymerase chain reaction (RT-qPCR). Skin biopsy was performed. Histopathologic findings revealed subepidermal blister formation, and it showed epidermal focal necrosis, red cell and netrophils in the blisters. The dermatopathology featured an infiltration of a variety of lymphocytes and neutrophils surrounding the vessels and accessory. Presence of lymphocyte cells' nucleus with atypia [Figure 2]. Immunohistochemistry showed CD2(+++), CD3(+++), CD4(+), CD5(++), CD7(+), CD8(±), CD20(−), CD30(−), CD56(+), ki67(30%+), Gr-B(+++) [Figure 3].{Figure 1}{Figure 2}{Figure 3}

Treatment and follow-up

The patient was treated with subcutaneous injections of interferon alpha (IFN-α) 50 ug every other day. Prednisone (40mg/d) for 1 week. At last, the patient's temperature fell down to normal level and the skin lesions were also gradually disappeared, edema was significantly reduced, and he was discharged from the hospital; no more detail information about the disease was available.


The immunohistochemical study of our patient showed CD2(+++), CD3(+++), CD4(+), CD5(++), CD7(+), CD8(±), CD20(−), CD30(−), CD56(+), ki67(30%+), Gr-B(+++); EBV-DNA level was positive, detected by RT-qPCR. Based on the clinical, pathologic, and immunohistochemical findings, a diagnosis of HV-like cutaneous T-cell lymphoma was made.

HV-like cutaneous T-cell lymphoma is most common in children, but can also be seen in adults, with more cases reported in Asia and Latin America.[5] Up to now, limited researches about HV-like cutaneous T-cell lymphoma in boys were reported in the literature due to its rarity.[6] This disease requires differential diagnosis with HV. HV is a rare chronic photodermatitis of childhood characterized by recurrent vacciniforme vesicles, necrotic ulcers, and scars on sun-exposed areas. Unlike typical HV, cutaneous lesions may also occur on nonexposed sites and minimal erythema dose phototesting is nonspecific. Lesion symptoms such as fever, hypersensitivity to mosquito, lymphadenopathy, hepatosplenomegaly, anemia, and leukopenia may develop. Our patient with EBV-associated cutaneous T/NK-cell proliferative disorders is characterized by similar clinicopathological features, including subcutaneous lymphoma associated with HV-like vesiculopapular eruptions. At present, the etiology and pathogenesis of HV-like cutaneous T-cell lymphoma are still unclear, which may be related to the chronic active infection or latent infection of EBV and the hypersensitivity reaction caused by mosquito bites.[7]

Treatment for patients of HV-like cutaneous T-cell lymphoma is not satisfactory. Immunosuppressive therapy, such as systemic corticosteroids, has been used, but the disease usually flares after decreasing the dose.[8] However, our patient was successfully treated with IFN-α and prednisone. After 2 months of follow-up, no obvious eruptions continued. This evidence highlights the therapeutic potential of antivirus treatment for HV-like cutaneous T-cell lymphoma. Most reported cases with EB virus infection happened in East Asia and Latin America, such as Japan, Korea, Taiwan, and Mexico. It has been suggested that the disease was induced by mosquito biting in genetic susceptible population, who have been infected with EB virus.[9],[10] However, the pathogenic genes and how EB virus induces lymphoma after mosquito bites need to be further studied. We hope that the investigation may provide some valuable data and experience in this special field.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


This study was supported by a grant from the National Natural Science Foundation of China (No. 81102066) and the Guangdong Science and Technology Project (No. 2016A020215223).

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


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