Photodermatol Photoimmunol Photomed 2017 Sep 20;33(5):287-290. Epub 2017 Jun 20.

Department of Dermatology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Surg Pathol Clin 2017 Jun 18;10(2):429-453. Epub 2017 Mar 18.

Haematological Malignancy Diagnostic Services (HMDS), Level 3, Bexley Wing, St James's University Hospital, Leeds LS9 7TF, UK. Electronic address:

Epstein-Barr virus (EBV)-associated lymphoproliferations involving the skin are a rare but important group of diseases with a broad spectrum of behavior, ranging from self-limiting spontaneously resolving disorders to highly aggressive malignancies. They may be of B, T, or natural killer (NK) cell type and include EBV-positive mucocutaneous ulcer, lymphomatoid granulomatosis, EBV-positive diffuse large B-cell lymphoma, hydroa vacciniforme-like lymphoproliferative disorder, and extranodal NK/T-cell lymphoma of nasal type. Recognition and distinction of these entities is important in view of their differing prognoses and treatments. Read More

Am J Dermatopathol 2017 Apr 4. Epub 2017 Apr 4.

Department of Dermatovenereology, West China Hospital, Sichuan University, Sichuan, China.

Hydroa vacciniforme-like lymphoma shows a marked predilection for the pediatric population, especially of Latin America and Asia. Here, we present 2 pediatric patients from Tibet of China, which is the first report happened in this area. Read More

Am J Dermatopathol 2017 Feb 15. Epub 2017 Feb 15.

Departments of *Dermatopathology, and †Dermatology, Universidade Federal do Pará, Brazil.

Hydroa vacciniforme (HV)-like lymphoma is a rare, usually fatal Epstein-Barr virus-driven lymphoproliferative disease affecting children from Asia, Mexico, and South America. Cutaneous manifestations imitate HV, a benign photodermatosis in which systemic symptoms are not observed, and spontaneous regression occurs later in adolescence or young adulthood. We report a case of HV-like lymphoma in a 12-year-old girl, descendent from an ancient Amazon indigenous tribe that, as far as we know, represents the second Brazilian case ever reported in the medical literature. Read More

Int J Hematol 2017 Apr 16;105(4):406-418. Epub 2017 Feb 16.

Department of Hematology/Oncology, Osaka Medical Center and Research Institute for Maternal and Child Health, 840 Murodo, Izumi City, Osaka, 594-1101, Japan.

Chronic active Epstein-Barr virus infection (CAEBV) is a prototype of the EBV-associated T- or NK-cell lymphoproliferative diseases, which also include hypersensitivity to mosquito bites and severe-type hydroavacciniforme. The manifestations of CAEBV are often self-limiting with minimum supportive care or only prednisolone and cyclosporine A with or without etoposide. However, allogeneic hematopoietic stem cell transplantation (HSCT) is the only cure, without which patients with CAEBV die within several years. Read More

Zhonghua Bing Li Xue Za Zhi 2016 Dec;45(12):817-821

Department of Pathology, Beijing Friendship Hospital Capital Medical University, Beijing 100050, China.

In recent years, there are increasing articles concerning Epstein-Barr virus associated lymphoproliferative disorder (EBV+ LPD), and the name of EBV+ LPD is used widely. However, the meaning of EBV+ LPD used is not the same, which triggered confusion of the understanding and obstacles of the communication. In order to solve this problem. Read More

Semin Diagn Pathol 2017 Jan 7;34(1):60-75. Epub 2016 Dec 7.

Hematopathology, National Cancer Institute (NCI), Bethesda, MD, USA.

This article will focus on the cutaneous lymphoproliferative disorders associated with EBV, with an emphasis on the upcoming changes in the revised 4th Edition of the WHO classification of tumors of the hematopoietic system, many of which deal with cutaneous disorders derived from NK-cells or T-cells. Extranodal NK/T-cell lymphoma usually presents in the upper aerodigestive tract, but can involve the skin secondarily. EBV-associated T- and NK-cell lymphoproliferative disorders (LPD) in the pediatric age group include the systemic diseases, chronic active EBV infection (CAEBV) and systemic EBV+ T-cell lymphoma of childhood. Read More

Clin Dermatol 2016 Nov - Dec;34(6):724-735. Epub 2016 Jul 12.

University of Connecticut School of Medicine, Department of Dermatology, Farmington, CT.

Photodermatoses are a group of skin disorders caused by abnormal reaction to ultraviolet radiation. Photodermatoses are divided into four groups: (1) immunologically mediated photodermatoses; (2) chemical- and drug-induced photodermatoses; (3) photoaggravated dermatoses; and (4) hereditary photodermatoses. This contribution discusses differences in the approach and diagnosis of pediatric and adult patients with suspected photodermatoses, focusing on immunologically mediated photodermatoses and chemical- and drug-induced photodermatoses. Read More

Acta Derm Venereol 2017 03;97(3):379-380

Department of Dermatology, Venereology and Allergology, University Hospital Würzburg, Josef-Schneider-Straße 2, DE-97080 Würzburg, Germany.

QJM 2016 Oct 9;109(10):697-698. Epub 2016 Aug 9.

Department of Dermatology, The First Affiliated Hospital, College of Medicine, Zhejiang University, No. 79, Qingchun Road, Hangzhou 310003, Zhejiang, People's Republic of China. email:

J Dermatol Sci 2016 Dec 3;84(3):358-359. Epub 2016 Oct 3.

Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 700-8558, Okayama, Japan. Electronic address:

J Cutan Pathol 2016 Dec 9;43(12):1102-1111. Epub 2016 Sep 9.

Department of Dermatology, College of Medicine, National Cheng Kung University and Hospital, Tainan, Taiwan.

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. Read More

Pan Afr Med J 2016 9;23:71. Epub 2016 Mar 9.

Hospital University Hassan II, Department of Dermatology, Faculty of Medicine, Fez, Morocco.

Clin Infect Dis 2016 Jul 11;63(1):41-7. Epub 2016 May 11.

Laboratorie of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases.

Background: Most patients infected with Epstein-Barr virus (EBV) are asymptomatic, have nonspecific symptoms, or have self-limiting infectious mononucleosis. EBV, however, may result in severe primary disease or cancer.

Methods: We report EBV diseases associated with GATA2 deficiency at one institution and describe the hematology, virology, and cytokine findings. Read More

Leuk Lymphoma 2017 Jan 9;58(1):53-63. Epub 2016 May 9.

a Department of Pathology , Seoul National University Hospital, Seoul National University College of Medicine , Seoul , South Korea.

Epstein-Barr virus-positive T/NK-cell lymphoproliferative diseases (EBV-T/NK-LPDs) include several overlapping EBV-related conditions with variably aggressive courses. For prognostic categorization, we retrospectively analyzed 42 EBV-T/NK-LPD cases. Male (79% [33/42]), young (≤40 years; 83% [35/42]) patients and T-cell lineage (81% [34/42]; CD8/CD4 = 1. Read More

JAAD Case Rep 2015 Mar 6;1(2):63-5. Epub 2015 Feb 6.

Department of Dermatology, American University of Beirut Medical Center, Beirut, Lebanon.

J Dermatol Sci 2016 Jun 5;82(3):153-9. Epub 2016 Mar 5.

Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama 700-8558, Japan. Electronic address:

Background: Epstein-Barr virus (EBV)-associated T/natural killer (NK)-lymphoproliferative disorders (LPDs) include hydroa vacciniforme (HV) and hypersensitivity to mosquito bites (HMB). The pathomechanisms of these diseases are still unclear.

Objective: To understand the inflammatory process, we examined EBV reactivation markers, BZLF1 and BDRF1 mRNA in the tissue and blood from patients with EBV-associated T/NK-LPDs. Read More

Br J Haematol 2016 May 15;173(4):573-81. Epub 2016 Mar 15.

Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, University of Pennsylvania, Philadelphia, PA, USA.

Mature T/Natural killer (NK)-cell neoplasms of children and the young adolescent population exhibit higher prevalence in Central and South American and Asian populations and many are associated with Epstein-Barr virus (EBV). They are represented in large part by extranodal T/NK cell lymphomas- nasal-type or extra nasal-type, chronic lymphoproliferative disorders of T/NK cells or chronic active EBV disease, systemic EBV-positive lymphoproliferative disorders of childhood, hydroa vacciniforme-like lymphoma, hepatosplenic T-cell lymphoma and primary cutaneous gamma/delta T-cell lymphoma among others. Many T/NK cell neoplasms in this age group are derived from cells of the innate immune system, in contrast to adults where they are predominantly from the adaptive immune system. Read More

Ann Lab Med 2016 May;36(3):268-70

Department of Laboratory Medicine & Genetics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Am J Dermatopathol 2016 May;38(5):e57-9

Servicio de Patología, Hospital Metropolitano, Quito, Ecuador.

Hydroa vacciniforme-like cutaneous lymphoma is a very rare Epstein-Barr virus positive peripheral T-cell lymphoma affecting Asian and Hispanic children and young adults with a defective cytotoxic immune response to EBV predisposing to the development of the disease. We report on 2 Ecuadorian patients with papulovesicular and ulcerated crusted lesions on the face, upper and lower extremities and abdomen, with aggressive clinical course and, in one case, a fatal outcome. The histological and molecular profiles (immunohistochemistry and in situ hybridization) established a diagnosis of hydroa vacciniforme-like Epstein-Barr virus-encoded small RNAs + cutaneous T-cell lymphoma in both cases. Read More

Clin Case Rep 2016 Feb 6;4(2):120-2. Epub 2015 Dec 6.

Dermatology Service KK Women's & Children's Hospital Singapore Singapore.

Hydroa vacciniforme is a photosensitivity disorder characterized by the childhood onset of necrotic vesiculopapules on exposed areas. We present a case of a 10-year-old boy with a 1-year history of papular lesions over his face. In this report, we discuss the in-depth histology of hydroa vacciniforme, and the management options. Read More

Ann Dermatol 2015 Dec 7;27(6):789-91. Epub 2015 Dec 7.

Department of Dermatology, Ajou University School of Medicine, Suwon, Korea.

Am J Dermatopathol 2016 Jan;38(1):20-5

*Centre for Dermatology & Dermatopathology and School of Medicine, Universidad Nacional Autónoma de México/Hospital General de México "Eduardo Liceaga," México, DF, Mexico; and †Research Unit Dermatopathology, Department of Dermatology, Medical University of Graz, Graz, Austria.

Hydroa vacciniforme-like lymphoma is a recently recognized cutaneous T-cell lymphoma associated with Epstein-Barr virus. The disease is observed in children of Latin American or Asian ethnicity. The authors report the clinical, histopathological, and immunophenotypical features of 9 new Mexican patients (M:F = 2:1; mean age, 14. Read More

Br J Dermatol 2015 Nov 8;173(5):1266-70. Epub 2015 Sep 8.

Department of Dermatology, CHU Caen, Université de Caen Basse Normandie, 14000, Caen, France.

Chronic active Epstein-Barr virus infection (CAEBV) is characterized by chronic infectious mononucleosis-like symptoms associated with very high viral load, as assessed by quantitative polymerase chain reaction. We present an unusual case in a French woman who was followed up over 25 years with cutaneous and sinus lymphoproliferation. This white woman presented with a long history of recurrent cutaneous necrotic papules of the skin, which started during childhood and healed spontaneously with depressed scars. Read More

Br J Dermatol 2015 Sep 22;173(3):801-5. Epub 2015 Jul 22.

Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan.

Hydroa vacciniforme (HV) is a rare photodermatosis that mainly affects children and manifests as vesiculopapular eruptions in sun-exposed areas without systemic symptoms. HV-like lymphoma (HVLL) is one of the Epstein-Barr virus (EBV)-associated lymphoproliferative disorders (LPD) of childhood. Its diagnosis is based on monoclonal T-cell proliferation; however, its degree of malignancy is controversial owing to its variable prognosis. Read More

J Eur Acad Dermatol Venereol 2015 Sep 25;29(9):1696-709. Epub 2015 Feb 25.

Department of Dermatology, University Hospital Zürich, Zürich, Switzerland.

Primary cutaneous lymphomas (CL) in children is rare. Only a few studies focused specifically on paediatric CL and therefore little is known whether primary CL in children are similar to or different from their adult counterparts with respect to the clinicopathological presentation, behaviour and prognosis. An extensive literature search using PubMed/MEDLINE from January 1995 through July 2014 was undertaken for articles reporting cases of paediatric CL. Read More

Br J Dermatol 2015 Jan;172(1):5-6

James P. Wilmot Cancer Center, University of Rochester School of Medicine, 601 Elmwood Avenue, Rochester, NY, 14642, U.S.A.

Int J Dermatol 2015 Mar 16;54(3):e84-5. Epub 2014 Dec 16.

Department of Dermatology, Tokyo Medical University, Tokyo, Japan.

J Am Acad Dermatol 2015 Jan;72(1):21-34; quiz 35-6

Cooper Medical School of Rowan University, Camden, New Jersey; Perelman School of Medicine at the Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania.

Epstein-Barr virus (EBV) was the first human virus to be associated with oncogenesis. Over the past few decades, cumulative research has revealed that latent EBV infection may be implicated in the pathogenesis of a heterogeneous group of lymphoproliferative disorders and malignancies occurring in both immunocompetent and immunocompromised hosts. Many of these diseases have either primary or secondary cutaneous manifestations. Read More

J Am Acad Dermatol 2015 Jan;72(1):1-19; quiz 19-20

Cooper Medical School of Rowan University, Camden, New Jersey; Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania.

Epstein-Barr virus (EBV) is a ubiquitous virus that has been implicated in a wide range of human diseases, many of which have mucocutaneous manifestations. As a member of the herpesviridae family, EBV causes lifelong infection by establishing latency in B lymphocytes. An intact immune response is critical in preventing progression of EBV disease, and the clinical manifestations of infection are dependent on the intricate relationship between virus and host immune system. Read More

J Cutan Pathol 2015 Mar 8;42(3):229-31. Epub 2014 Dec 8.

Department of Dermatology, Gangnam Severance Hospital, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.

BMJ Case Rep 2014 Nov 24;2014. Epub 2014 Nov 24.

Department of Dermatology and Allergy Centre, Odense University Hospital, Odense, Denmark.

Hydroa vacciniforme is one of the rarest forms of photosensitivity disorders of the skin. Effective treatment options are scarce and mainly constitute of strict sun protection. Lately, hydroa vacciniforme has been associated with Epstein-Barr virus infection. Read More

MMW Fortschr Med 2014 Jun;156(12)

Int J Clin Exp Pathol 2014 15;7(9):6403-8. Epub 2014 Aug 15.

Department of Hematology, The First Affiliated Hospital of Nanjing Medical University, Jiangsu Province Hospital Nanjing 210029, China.

Hydroa vacciniforme-like lymphoma (HVLL) is an extremely rare lymphoma described in children that occurs mainly in Asia and Latin American countries. It is an Epstein-Barr virus (EBV)-positive lymphoproliferative disease (LPD) characterized by a monoclonal proliferation of T or NK cells. In this study, we report the clinical and pathological features of two Chinese patients with HVLL showed T-cell phenotype expressing CD4. Read More

Br J Dermatol 2015 Jan 10;172(1):56-63. Epub 2014 Dec 10.

Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama, 700-8558, Japan.

Background: Epstein-Barr virus (EBV)-associated T/natural-killer lymphoproliferative disorders form a group of diseases that includes classical and systemic hydroa vacciniforme (HV) and hypersensitivity to mosquito bites (HMB). Patients with systemic HV (sHV) and HMB often have a poor prognosis, although little is known about the prognostic factors.

Objectives: To elucidate the prognostic factors of HV and HMB. Read More

Pediatr Dermatol 2015 Jan-Feb;32(1):e39-41. Epub 2014 Sep 10.

Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.

We herein report two patients with hydroa vacciniforme and concomitant ophthalmic involvement, primarily in the form of keratoconjunctivitis leading to corneal opacity. Awareness of this occasional manifestation may help to prevent severe complications. Read More

Int J Clin Exp Pathol 2014 15;7(8):4991-9. Epub 2014 Jul 15.

Department of Pathology, Cancer Institute and Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College Beijing, 100021, China.

A new category, "EBV positive T-cell lymphoproliferative disorders (LPD) of childhood", was proposed in the 2008 World Health Organization's (WHO) classifications of lymphoma. This series of lymphoproliferative disorders is rare. There are two major types of this series of disorders: systemic EBV positive T-cell LPD of childhood and hydroa vacciniforme-like lymphoma (HVLL). Read More

Am J Hematol 2014 Dec 26;89(12):1160-1. Epub 2014 Sep 26.

Department of Oncology and Radiotherapy, Hospital Nacional Edgardo Rebagliati Martins, Lima, Peru.

Am J Dermatopathol 2015 Jan;37(1):20-5

*Department of Pathology, Division of Dermatopathology, Medical College of Wisconsin, Milwaukee, WI; and †Department of Pathology and Dermatology, Division of Dermatopathology, Hospital Obrero, La Paz, Bolivia.

Hydroa vacciniforme-like lymphoma (HVL) is a rare cutaneous T-cell lymphoma that is usually seen in children of Hispanic or Asian origin. Association between chronic latent Epstein-Barr virus infection in both hydroa vacciniforme (HV) and HVL has been demonstrated and has recently been categorized by the World Health Organization as one of the Epstein Barr virus-positive lymphoproliferative disorders of childhood. Patients with HVL present with a cutaneous rash characterized by edema, blisters, ulcers, and scars mainly seen on the face and extremities that mimic HV; however, unlike in HV, the lesions tend to be extensive and deeper and are associated with severe scarring, necrosis, and systemic manifestations. Read More

Dermatol Online J 2014 Aug 17;20(8). Epub 2014 Aug 17.

Baylor College of Medicine.

Hydroa vacciniforme (HV) is a rare photodermatosis characterized by a recurrent vesiculopapular eruption with varioliform scarring. The pathogenesis remains unknown. Herein we present a case to emphasize the importance of recognizing this condition and its clinical mimickers, which include other photodermatoses and lymphoma. Read More

Clin Cancer Res 2014 Oct 12;20(19):5075-84. Epub 2014 Aug 12.

Department of Virology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Purpose: Epstein-Barr virus (EBV) infects not only B cells but also T cells and natural killer (NK) cells, and T- and NK-cell lymphoproliferative diseases (T/NK-LPD) that are refractory to conventional chemotherapies may develop. To identify a molecular-targeted therapy for EBV-associated T/NK-LPDs, we investigated whether CC chemokine receptor 4 (CCR4) was expressed on EBV-infected T and/or NK cells and whether a humanized anti-CCR4 monoclonal antibody, mogamulizumab, was effective.

Experimental Design: CCR4 expression was examined in various cell lines. Read More

J Pediatr Hematol Oncol 2015 May;37(4):e223-6

Department of *Pediatrics †Dermatology ‡Pathology, Christian Medical College, Vellore, India.

We report a 14-year-old Indian boy who presented with a history of weight loss, fever, facial edema, and a relapsing papulovesicular eruption on the face and limbs for 1 year. Histopathology of the skin showed dense lymphoid infiltrate from dermis to subcutaneous fat. Immunohistochemistry of this lymphoid infiltrate was CD3, CD8, CD56, CD57, Granzyme B, TIA, and Epstein Barr virus LMP1. Read More

Int J Clin Exp Pathol 2014 15;7(5):2430-7. Epub 2014 Apr 15.

Department of Pathology, Chi-Mei Medical Center Tainan, Taiwan ; Taipei Medical University and National Taiwan University Taipei, Taiwan.

Epstein-Barr Virus (EBV) is a herpesvirus usually infecting B-cells but may occasionally infect T- or natural killer (NK)-cells. EBV-associated T- or NK-cell lymphoproliferations represent a continuous spectrum of diseases ranging from asymptomatic infection, infectious mononucleosis (IM), to clonal and malignant lymphoproliferations including systemic EBV-positive T/NK-cell lymphoproliferative disease (EBV-T/NK-LPD) of childhood and hydroa-vacciniforme-like lymphoma of the skin. The clonal diseases are more prevalent in East Asia and exhibit overlapping clinical and pathological features with chronic active EBV infection. Read More

Dermatol Clin 2014 Jul 24;32(3):345-53, viii. Epub 2014 Apr 24.

Department of Dermatology, Faculty of Medicine, Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkoknoi, Bangkok 10700, Thailand. Electronic address:

Hydroa vacciniforme (HV) and solar urticaria (SU) are uncommon immunologically mediated photodermatoses. HV occurs almost exclusively in children, usually beginning in childhood and remitting spontaneously by adolescence. Association with chronic Epstein-Barr virus infection has been reported in HV, which raises the possibility of lymphoproliferative disorders in these patients. Read More

J Paediatr Child Health 2014 Mar;50(3):244, 247

Dermatology Department, Centro Hospitalar do Porto, Porto, Portugal.

Nagoya J Med Sci 2013 Aug;75(3-4):169-79

Ubiquitous Epstein-Barr virus (EBV) infects not only B cells but also T and NK cells, and is associated with various lymphoid malignancies. The spectrum of EBV-associated lymphoid malignancies is expanding from Burkitt lymphoma to the newly defined systemic EBV+ T cell lymphoproliferative disease of childhood and hydroa vacciniforme-like lymphoma. However, some EBV-associated malignancies are not defined well and overlap other diseases. Read More

J Dermatol 2014 Apr 15;41(4):360-2. Epub 2014 Mar 15.

Department of Dermatology, Shizuoka Municipal Shimizu Hospital, Shizuoka, Japan.

Pediatr Int 2014 Apr;56(2):159-66

Department of Infectious Diseases, National Research Institute for Child Health and Development, Tokyo, Japan.

Epstein-Barr virus (EBV) infection is usually asymptomatic and persists lifelong. Although EBV-infected B cells have the potential for unlimited proliferation, they are effectively removed by the virus-specific cytotoxic T cells, and EBV-associated lymphoproliferative disease develops only in immunocompromised hosts. Rarely, however, individuals without apparent immunodeficiency develop chronic EBV infection with persistent infectious mononucleosis-like symptoms. Read More

J Am Acad Dermatol 2014 Apr 9;70(4):724-35. Epub 2014 Feb 9.

Department of Pathology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.

Mycosis fungoides is the prototype of primary cutaneous T-cell lymphoma and is more common in the West than in the East, whereas nonmycotic primary cutaneous T-cell lymphoma is more frequent than mycosis fungoides among Asians. Nonmycotic primary cutaneous T-cell lymphomas comprise several categories of neoplasms and might pose diagnostic challenges because of the rarity of these lesions and overlapping features among certain entities. The authors recommend diagnostic approaches including histopathological evaluation, immunohistochemical markers, and ancillary studies. Read More