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Adv Exp Med Biol 2022 ;1367:369-381

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

Photodermatosis is an abnormal skin inflammatory reaction to light. The major classifications of photodermatoses are idiopathic photodermatoses, photodermatoses due to exogenous or endogenous agents, photo-exacerbated dermatoses, and photosensitive genodermatoses. In this chapter, we focus on idiopathic photodermatoses and drug-related photodermatoses and emphasize on the epidemiology and immunogenetic backgrounds. Read More

Int J Hematol 2022 Mar 10. Epub 2022 Mar 10.

Department of Hematology, Tohoku University Hospital, 1-1, Seiryo-machi, Aoba-ku, Sendai, Miyagi, 980-8574, Japan.

Adult-onset EBV-associated T-cell and NK-cell lymphoproliferative disorders (EBV-T/NK-LPDs) often progress rapidly, and require allogeneic stem cell transplantation early in the course of treatment. Unrelated cord blood transplantation (UCBT) is a readily available option for patients without HLA-matched donors. We retrospectively analyzed the outcomes of 12 UCBT in adult patients with chronic active EBV infection (CAEBV, n = 8), EBV-positive hemophagocytic lymphohistiocytosis following primary EBV infection (n = 2), hydroa vacciniforme-like lymphoproliferative disorder (n = 1), and systemic EBV-positive T-cell lymphoma of childhood (STCLC, n = 1). Read More

Pediatr Dermatol 2022 Feb 22. Epub 2022 Feb 22.

Department of Pathology, Molecular Medicine and Cancer Research Center, Chongqing Medical University, Chongqing, China.

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. Read More

J Cutan Med Surg 2022 Feb 15:12034754221077694. Epub 2022 Feb 15.

Department of Pathology, Sainte-Justine University Hospital Center, University of Montreal, Montreal, Quebec, Canada.

Background: Cutaneous hematologic malignancies are rare in children, and the literature about them is still sparse.

Objective: The purpose of our study was to report our experience with pediatric cases of cutaneous hematologic disorders and describe their clinical and histological features.

Methods: Data were retrospectively collected from the histopathologic database of the CHU Sainte-Justine, University of Montreal, Montreal, Canada. Read More

Rev Med Virol 2022 Feb 4:e2328. Epub 2022 Feb 4.

Department of Microbiology, School of Medicine, Alborz University of Medical Sciences, Karaj, Iran.

EBV is a ubiquitous virus that infects nearly all people around the world. Most infected people are asymptomatic and do not show serious sequelae, while others may develop Epstein-Barr virus (EBV)-positive T and NK-cell lymphoproliferations characterised by EBV-infected T or NK cells. These disorders are more common in Asian and Latin American people, suggesting genetic predisposition as a contributing factor. Read More

J Cutan Pathol 2022 Jun 26;49(6):560-564. Epub 2022 Jan 26.

Oral Pathology, Department of Stomatology, Public Oral Health, and Forensic Dentistry, Ribeirão Preto Dental School (FORP/USP), University of São Paulo, Ribeirão Preto, Brazil.

The primary cutaneous (PC) CD8+ T-cell lymphoproliferative disorders (LPDs) comprise clinically and histopathologically heterogeneous entities including mycosis fungoides, lymphomatoid papulosis, hydroa-vacciniforme-like LPD, subcutaneous panniculitis-like T-cell lymphoma (TCL), PC acral CD8+ TCL, PC CD8+ aggressive epidermotropic cytotoxic TCL, and PC peripheral TCL, not otherwise specified (PTCL-NOS). We describe a 33-year-old man who presented with progressive facial swelling and lower lip involvement 1 year ago. Microscopy revealed an atypical small to medium-sized lymphoid proliferation exhibiting perivascular accentuation, adnexotropism, and apoptotic cell debris, without surface epithelium involvement. Read More

Pediatr Dermatol 2021 Sep 24;38(5):1387-1388. Epub 2021 Sep 24.

Department of Dermatology, West China Hospital Sichuan University, Chengdu, China.

Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is characterized by vesiculopapular eruptions on sun-exposed and sometimes unexposed skin. Though ocular involvement in HVLPD is rare, it may present with conjunctivitis, corneal opacities, uveitis, and interstitial keratitis. We report a case of a 7-year-old boy with HVLPD, whose ophthalmic symptoms were neglected for over 2 years, who developed anterior uveitis and corneal nebulae without vision impairment. Read More

J Med Cases 2020 Nov 23;11(11):366-369. Epub 2020 Sep 23.

Department of Haematology, Sir Charles Gairdner Hospital, Hospital Ave, Nedlands, Western Australia 6009, Australia.

Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a rare Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disorder (LPD) which primarily affects children from Latin America and Asia. Typical features include vesicles and ulceration in sun-exposed areas which may be accompanied by systemic symptoms such as fever, lymphadenopathy and hepatosplenomegaly. We report a 73-year-old man diagnosed with HV-LPD in the context of zanubrutinib (oral Bruton tyrosine kinase (BTK)-inhibitor) treatment for chronic lymphocytic leukemia (CLL). Read More

J Oral Pathol Med 2021 Jul 29;50(6):530-539. Epub 2021 Jun 29.

Departamento de Patología, Medicina y Cirugía Oral, Facultad de Estomatología, Universidad Peruana Cayetano Heredia, Lima, Perú.

Background: Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a chronic Epstein-Barr virus (EBV)-positive lymphoproliferative disease which may either present as an indolent condition or progress to a systemic T-cell lymphoma.

Methods: All HVLPD diagnosed over a 10-year period were retrieved, and clinical data regarding sex, age, oral and systemic manifestations, and clinical follow-up were obtained. Immunohistochemistry was done in order to characterize the lymphoid cells, and in situ hybridization was used to demonstrate the presence of EBV. Read More

Ann Dermatol 2021 Jun 4;33(3):222-227. Epub 2021 May 4.

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

Background: Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is rare Epstein-Barr virus (EBV)-associated disease. The classic form of HVLPD is a self-resolving disease, whereas the systemic form can progress to malignant lymphoma, resulting in fatal outcomes. However, the prognostic factors remain unclear. Read More

Surg Pathol Clin 2021 Jun 29;14(2):177-194. Epub 2021 Apr 29.

Department of Hematopathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 72, Houston, TX 77030, USA.

Primary cutaneous T-cell lymphomas pose a diagnostic challenge for dermatopathologists, hematopathologists, and general surgical pathologists. Recognition of gamma/delta phenotype in cutaneous T proliferations has been enhanced by the availability of antibodies against TCRgamma and delta for immunohistochemistry. Thus, reporting gamma/delta phenotype in a cutaneous T-cell lymphoid proliferation may indicate a significant change in therapy and a challenge for dermatologists and oncologists who treat these patients. Read More

J Dermatol 2021 Sep 13;48(9):1315-1326. Epub 2021 May 13.

Department of Oncology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.

Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a rare cutaneous disease associated with Epstein-Barr virus infection. We retrospectively analyzed the clinical presentation, histopathological characteristics, and prognostic study of HV-LPD in 24 Chinese patients. All patients presented with recurrent papulovesicular and necrotic eruptions on the face, neck, and extremities, with 11 showing systemic symptoms. Read More

Indian J Dermatol Venereol Leprol 2021 Apr 12:1-5. Epub 2021 Apr 12.

Department of Dermatologic Surgery, Dermatologic Institute of Jalisco Dr. José Barba Rubio, Guadalajara, Mexico.

We report the clinical and histopathological features of hydroa vacciniforme-like lymphoproliferative disorder in five indigenous and Mestizo children. All the children resided at higher altitudes, experiencing maximal solar exposure. All cases presented with prurigo along with Epstein-Barr virus infection. Read More

An Bras Dermatol 2021 May-Jun;96(3):388-390. Epub 2021 Mar 24.

Hospital Universitario San Ignacio, Bogotá, Colombia.

BMC Infect Dis 2021 Jan 6;21(1):17. Epub 2021 Jan 6.

Center of Infectious Diseases, Beijing Ditan Hospital, Capital Medical University, 8 Jingshun East Street, Chaoyang District, Beijing, 100015, China.

Background: Hydroa Vacciniforme-like Lymphoproliferative Disorder (HV-LPD) is the name given to a group of Epstein-Barr virus (EBV)-associated diseases. It resembles hydroa vacciniforme (HV), the rarest form of photosensitivity, and is a T-cell disorder associated with an Epstein-Barr virus infection. The majority of diagnosed cases occur in East Asia and South America. Read More

Int J Mol Sci 2020 Dec 7;21(23). Epub 2020 Dec 7.

Department of Dermatology, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan 704, Taiwan.

Hydroa vacciniforme (HV) is a rare form of photosensitivity disorder in children and is frequently associated with Epstein-Barr virus (EBV) infection, whereas HV-like lymphoproliferative disorders (HVLPD) describe a spectrum of EBV-associated T-cell or natural killer (NK)-cell lymphoproliferations with HV-like cutaneous manifestations, including EBV-positive HV, atypical HV, and HV-like lymphoma. Classic HV occurs in childhood with papulovesicules on sun-exposed areas, which is usually induced by sunlight and ultraviolet irradiation, and mostly resolves by early adult life. Unlike classic HV, atypical or severe HV manifests itself as recurrent papulovesicular eruptions in sun-exposed and sun-protected areas associated occasionally with facial edema, fever, lymphadenopathy, oculomucosal lesions, gastrointestinal involvement, and hepatosplenomegaly. Read More

Photodermatol Photoimmunol Photomed 2021 05 8;37(3):195-197. Epub 2020 Dec 8.

Photodermatosis Service and Laser Unit, Division of Dermatology, Rabin Medical Center, Petah-Tikva, Israel.

Sci Rep 2020 11 9;10(1):19294. Epub 2020 Nov 9.

Department of Dermatology, Seoul National University College of Medicine, Seoul, Republic of Korea.

Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) is a rare Epstein-Barr virus (EBV)-associated lymphoproliferative disease. The disease course of HVLPD varies from an indolent course to progression to aggressive lymphoma. We investigated the characteristics of HVLPD in Korean patients. Read More

Cancers (Basel) 2020 Oct 30;12(11). Epub 2020 Oct 30.

Department of Allergy and Clinical Immunology, National Research Institute for Child Health and Development, Tokyo 157-8535, Japan.

Chronic active Epstein-Barr virus (EBV) infection (CAEBV) is a rare syndrome characterized by prolonged infectious mononucleosis-like symptoms and elevated peripheral blood EBV DNA load in apparently immunocompetent persons. CAEBV has been primarily reported in East Asia and Latin America, suggesting a genetic predisposition in its pathogenesis. In most cases of CAEBV, EBV induces proliferation of its unusual host cells, T or natural killer (NK) cells. Read More

Ocul Immunol Inflamm 2022 Feb 6;30(2):320-323. Epub 2020 Oct 6.

Department of Ophthalmology, Sichuan Provincial Corps Hospital, Chinese People's Armed Police Forces, Leshan, China.

Purpose: To report a case of ocular involvement associated with hydroa vacciniforme-like lymphoproliferative disorder (HVLPD).

Case Report: A 10-year-old HVLPD boy suffered conjunctivitis, interstitial keratitis, and anterior uveitis sequentially during the whole course. Interestingly, this case manifested initially only with ocular findings, which preceded 1 year earlier than the onset of cutaneous lesions. Read More

Int J Dermatol 2020 Dec 17;59(12):e452-e454. Epub 2020 Aug 17.

Department of Dermatology, Faculty of Medicine, Pamukkale University, Denizli, Turkey.

J Dermatol Sci 2020 Aug 9;99(2):128-134. Epub 2020 Jul 9.

Department of Dermatovenerology, West China Hospital, Sichuan University Chengdu, China. Electronic address:

Background: Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) encompasses a rare group of Epstein-Barr virus (EBV)-associated lymphoproliferative diseases.

Objective: To define the clinical and pathologic characteristics of HVLPD and to identify mutant genes that may be related to the development of HVLPD.

Methods: Clinical data and archived formalin-fixed, paraffin-embedded tissue were obtained from 19 patients. Read More

Acta Derm Venereol 2020 Jun 18;100(13):adv00192. Epub 2020 Jun 18.

Department of Dermatology, APHP, Hôpital Avicenne, FR-93000 Bobigny, France.

Int J Dermatol 2020 Aug 13;59(8):e290-e292. Epub 2020 May 13.

Department of Dermatology & Dermatological Surgery, Medical University of South Carolina, Charleston, SC, USA.

J Med Virol 2020 Apr 7. Epub 2020 Apr 7.

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

Epstein-Barr virus (EBV) DNA load in the blood increases in posttransplant lymphoproliferative disorders and chronic active EBV infection. In this report, we analyzed the EBV DNA load in the peripheral blood mononuclear cells (PBMCs) and plasma of patients with hydroa vacciniforme (HV) and/or hypersensitivity to mosquito bites (HMB) to understand the clinical significance of EBV DNA load. All 30 patients showed high DNA loads in the PBMCs over the cut-off level. Read More

Photodermatol Photoimmunol Photomed 2020 May 12;36(3):233-240. Epub 2020 Mar 12.

Department of Dermatology, Henry Ford Hospital, Detroit, Michigan.

Background: Previous studies at single academic institutions have identified variations in the prevalence of photodermatoses among racial groups. The purpose of the study was to compare the distribution of photodermatoses between Whites and Blacks at four academic medical centers in the USA.

Methods: A retrospective chart review was performed at four institutions' general dermatology clinics using diagnoses consistent with the International Classification of Disease (ICD), Ninth and Tenth Revisions, codes related to photodermatoses between August 2006 and August 2016. Read More

Indian J Dermatol 2019 Nov-Dec;64(6):493-496

Department of Dermatology, The Second Xiangya Hospital, Central South University, Changsha 410011, Hunan, China.

Two Chinese children presented with relapsing papules, vesicles, ulcers on the face and limbs with systemic symptoms being caused by mosquito bite. Lesion biopsy revealed atypical lymphocyte in the dermis and subcutis. Immunohistochemistry identified cells as positive for CD3, CD45RO, TIA-1 and EBER. Read More

Semin Diagn Pathol 2020 Jan 17;37(1):32-46. Epub 2019 Dec 17.

Institute of Pathology and Neuropathology and Comprehensive Cancer Center Tübingen, University Hospital Tübingen, Eberhard-Karls-University, Tübingen, Germany. Electronic address:

The spectrum of Epstein-Barr virus (EBV)-positive T and NK-cell lymphoproliferations is broad and ranges from reactive self-limited disorders to neoplastic processes with a fulminant clinical course. EBV plays an important role promoting lymphomagenesis, although the precise mechanisms remain elusive. EBV-positive lymphoproliferative disorders (LPD) are more common in East Asia (China, Japan, Korea and Taiwan), and Latin America suggesting a strong genetic predisposition. Read More

Leuk Lymphoma 2020 04 13;61(4):808-819. Epub 2019 Dec 13.

Laboratory of Pathology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA.

Epstein-Barr virus (EBV) normally infects B cells, but in some persons the virus infects T or NK cells. Infection of B cells can result in infectious mononucleosis, and the virus is associated with several B cell malignancies including Hodgkin lymphoma, Burkitt lymphoma, and diffuse large B cell lymphoma. Infection of T or NK cells with EBV is associated with extranodal NK/T cell lymphoma, aggressive NK-cell leukemia, systemic EBV-associated T-cell lymphoma, and chronic active EBV disease, which in some cases can include hydroa vacciniforme-like lymphoproliferative disease and severe mosquito bite allergy. Read More