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Viral-induced cutaneous T-cell lymphomas are an uncommon group of lymphoproliferative disorders characterized by a viral infection of T and natural killer (NK) cells. This group of cutaneous T-cell lymphomas is more commonly encountered in Asians and Native Americans from Central and South America compared with Western populations. Viral-associated lymphoproliferative disorders include a spectrum of entities that range from nonneoplastic lesions, such as chronic active Epstein-Barr virus infection and infective dermatitis to malignant diseases, such as extranodal NK/T-cell lymphoma, hydroa vacciniforme-like T-cell lymphoma, and adult T-cell leukemia/lymphoma. This review article will focus on hydroa vacciniforme-like lymphoproliferative disorder, extranodal NK/T-cell lymphoma, adult T-cell leukemia/lymphoma, lymphomatoid granulomatosis, and Epstein-Barr virus-positive mucocutaneous ulcers. We will review the pathogenesis of these conditions and the challenges of making a timely diagnosis in early-stage disease and discuss the common clinicopathologic manifestations, mutational landscape, and approaches to treat these highly aggressive and frequently lethal types of lymphoma., Competing Interests: Conflict of interest None disclosed., (Copyright © 2022 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Hydroa vacciniforme-like lymphoproliferative disorder (HVLLPD) is a rare Epstein-Barr virus (EBV)-related disease that is usually found in East Asians and Latin Americans., Objective: To report the characteristics of HVLLPD in Chinese patients., Methods: Retrospective analysis of patients with HVLLPD from a single institute., Results: A total of 41 patients were enrolled. All patients presented with papulovesicular lesions, mainly distributed on sun-exposed areas, with 26 patients showing systemic symptoms. Follow-up data were available for 20 patients, 16 patients were alive, and 4 patients died. Of the 4 deceased patients, 3 had taken a serum EBV DNA test that showed high viral loads. These 3 patients also received chemotherapy. Histopathology was characterized by dense proliferation of lymphocytes in the dermis. Angiotropism or angiodestruction was found in the majority of patients, whereas prominent cellular polymorphism was noticed in only 4 patients. All patients were positive for CD3, TIA1 cytotoxic granule associated RNA binding protein, and EBV-encoded RNA in situ hybridization., Limitations: This was a retrospective study., Conclusions: HVLLPD in Chinese patients showed indolent behavior in the majority of cases, which differed from the characteristics of HVLLPD in Latin Americans. Patients with high serum EBV DNA loads had an increased risk of their disease evolving into aggressive disease. Chemotherapy should not be considered as first-line treatment for most Chinese patients., (Copyright © 2019 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Hydroa vacciniforme (HV)-like lymphoma (HVL) is a rare and aggressive cutaneous T-cell lymphoma occurring mainly in children in Latin America and Asia. Chronic latent Epstein-Barr virus infection has been associated with both HV and HVL., Objective: We sought to evaluate the clinical presentation and histopathology of this rare cutaneous T-cell lymphoma., Methods: We reviewed the clinical, morphologic, and immunophenotypical features in 12 cases of HVL from Bolivia., Results: All 12 patients had skin lesions in both sun-exposed and nonsun-exposed areas, including edema, blistering, ulceration, and scarring, with a slowly progressive relapsing course. All 12 patients presented with systemic symptoms and showed a characteristic swelling of the nose and lips, and periorbital edema. Eight patients died an average of 5.3 months after initial diagnosis. Four patients remained alive with persistent disease. Histopathologic examination showed an atypical lymphocytic infiltrate with angiotropism and angiocentricity. The immunophenotype showed a cytotoxic T-cell (CD8(+)) profile. All cases were associated with Epstein-Barr virus infection and differed clinically from other forms of cutaneous T-cell lymphoma., Limitations: Only a limited number of cases were studied., Conclusions: This study confirms that HVL is a highly aggressive lymphoma, although some patients have a more indolent, chronic course., (Copyright © 2013 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Background: Hydroa vacciniforme (HV) is a rare, sporadic, idiopathic photodermatosis characterized by vesicles and crust formation after sunlight exposure. The lesions typically heal with vacciniform scarring., Objective: We identify and review the clinical features and follow-up data of Scottish patients with HV and report on the prevalence of this condition. This is the largest recent study of HV patients from a single center., Methods: In this retrospective study, patients with HV were identified by means of the diagnostic database from the Photobiology Unit, Dundee. Patients were contacted and details of clinical features, duration of disease, results of investigations, and treatment were recorded. At review, disease progress was assessed., Results: Between 1973 and 1997, 17 patients (9 males and 8 females) with a diagnosis of HV were investigated. Data from 15 patients showed a mean age at onset of 7.9 years (range, 1 to 16 years), with females (mean, 6.7 years; range, 2 to 12 years) having an earlier onset than males (mean, 8.7 years; range, 1 to 16 years). A bimodal age distribution was also identified with onsets between the ages of 1 and 7 years and 12 and 16 years. At review, spontaneous clearing had occurred in 9 patients (60%) with mean duration of disease being 9 years (range, 4 to 17 years). Males had longer disease duration (mean, 11 years; range, 5 to 17 years) than females (mean, 5 years; range, 4 to 7 years). Eight patients (53%) were sensitive in the UVA wave-band on monochromator phototesting, and 6 (40%) experienced papulovesicular lesions on repetitive broad-spectrum UVA irradiation. All patients received broad-spectrum sunscreens with variable results. Of the 5 patients treated with narrow-band UVB (TL-01) phototherapy, 3 reported beneficial results with an increase in tolerance to sunlight exposure and associated reduction in disease severity., Conclusion: The estimated prevalence of HV was at least 0.34 cases per 100,000 with an approximately equal sex ratio. Males had a later onset and longer duration of disease than females. Phototesting showed abnormal responses in the UVA wavebands in 53% of cases, whereas 60% of patients treated with prophylactic TL-01 phototherapy found it beneficial.

An 8-year-old Japanese girl had a 9-month history of a self-healing papulovesicular eruption on her face, scalp, and neck that resembled hydroa vacciniforme (HV). Histologically, there was a dense infiltration of small lymphocytic cells and scattered large atypical cells expressing CD30. Study of gene rearrangement showed no monoclonality in the infiltrating cells. To our knowledge, this is the second case of lymphomatoid papulosis with clinical features resembling HV. However, we also found descriptions in the literature of two other Japanese children with malignant lymphoma who both initially had clinical features resembling HV. These findings suggest that these cases of HV-like disease constitute a subset of lymphomatoid papulosis that is highly likely to progress to malignant lymphoma.

Hydroa vacciniforme is a rare, chronic photodermatosis with onset in childhood that is characterized by recurrent vesiculation, necrosis, and vacciniform scarring limited to sun-exposed skin. We report a case of nonvesicular hydroa vacciniforme in which only extensive crusting associated with hypertrophic scarring on sun-exposed skin was present. Multiple exposures to UVA, however, reproduced lesions with the typical clinical and histologic features of hydroa vacciniforme. Results of blood, urine, and fecal porphyrin studies were normal.

Hydroa vacciniforme is a rare, chronic, photosensitive disorder manifested in childhood by recurrent vesicles that heal with scarring. Reproduction of vesicles with repetitive UVA phototesting may be an important diagnostic aid. Recurrent eruption resulted in severe scarring of the face and a flexion contracture of the finger in our patient. The clinical features and laboratory evaluation of hydroa vacciniforme are reviewed.

Hydroa vacciniforme is a rare photosensitivity disorder with onset in childhood. The distinctive lesion is a vesicle which heals with scarring. We report a case of hydroa vacciniforme in which an abnormal minimal erythema dose to wavelengths of 322 to 370 nm within the ultraviolet A (UVA) range was demonstrated. Vesicles could be induced only with multiple exposures to UVA. Increased tolerance to UVA erythema was induced by multiple UVB exposures, although tanning was poor. Of note were the presence of several halo nevi and a history of the loss of ability to tan. The clinical features and appropriate laboratory evaluation of hydroa vacciniforme are reviewed.