Hydroa vacciniforme-like lymphoma

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Alberto Plate [2]

Synonyms and Keywords: Hydroa-like CTCL, HVLL


Hydroa vacciniforme-like lymphoma (HVLL) is an EBV-positive peripheral T-cell lymphoma (non-Hodgkin's lymphoma), that affects primarily pediatric patients and has a bad prognosis.[1] HVLL is associated with photosensitivity and insect bites.[2] Clinically, HVLL manifests with vacciniform vesicles, necrotic ulcers, and scars.[3] Some cases of Hydroa vacciniforme-like lymphoma express NK-cell surface receptors.[4]

Historical Perspective

HVLL reports date back to 1986;[5] however, it is not until 2008 that the WHO recognized HVLL as a separate entity from Hydroa vacciniforme and classified it under "EBV positive T-cell lymphoproliferative disorders of childhood".[2]



Histopathologic examination demonstrates lymphocytic infiltrate in the skin, that are characterized by:[6]

  • Angiotropism
  • Angiocentricity


Differential Diagnosis

Epidemiology and Demographics


HVLL occurs mainly in the pediatric population.[12] Although rare, it may present in adult population.[13]


In one study, there was difference in incidence when comparing men and women.[14]

Developing Countries

HVLL occurs in South America, Central America, and Asia.[12]

Risk Factors

Natural History, Complications, and Prognosis


History and Symptoms

Physical Examination

Exploratory Findings

  • EBV DNA in peripheral blood
  • EBV RNA in histological cuts of skin lesions with in situ hybridization
  • Tumor cells positive for EBV RNA[4]

Laboratory Findings

  • IgM positive for EBV



  1. 1.0 1.1 1.2 Barrionuevo C, Anderson VM, Zevallos-Giampietri E, Zaharia M, Misad O, Bravo F; et al. (2002). "Hydroa-like cutaneous T-cell lymphoma: a clinicopathologic and molecular genetic study of 16 pediatric cases from Peru". Appl Immunohistochem Mol Morphol. 10 (1): 7–14. PMID 11893040.
  2. 2.0 2.1 Swerdlow, Steven (2008). WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon, France: International Agency for Research on Cancer. ISBN 9789283224310.
  3. "Hydroa vacciniforme-like lymphoma: a case report and literature review" (PDF).
  4. 4.0 4.1 4.2 Xu Z, Lian S (2010). "Epstein-Barr virus-associated hydroa vacciniforme-like cutaneous lymphoma in seven Chinese children". Pediatr Dermatol. 27 (5): 463–9. doi:10.1111/j.1525-1470.2010.01094.x. PMID 20497358.
  5. Oono T, Arata J, Masuda T, Ohtsuki Y (1986). "Coexistence of hydroa vacciniforme and malignant lymphoma". Arch Dermatol. 122 (11): 1306–9. PMID 3490832.
  6. 6.0 6.1 Sangueza M, Plaza JA (2013). "Hydroa vacciniforme-like cutaneous T-cell lymphoma: clinicopathologic and immunohistochemical study of 12 cases". J Am Acad Dermatol. 69 (1): 112–9. doi:10.1016/j.jaad.2013.01.037. PMID 23541598.
  7. Wu YH, Chen HC, Hsiao PF, Tu MI, Lin YC, Wang TY (2007). "Hydroa vacciniforme-like Epstein-Barr virus-associated monoclonal T-lymphoproliferative disorder in a child". Int J Dermatol. 46 (10): 1081–6. doi:10.1111/j.1365-4632.2007.03102.x. PMID 17910721.
  8. "Hydroa vacciniforme-like lymphoma: a chronic EBV+lymphoproliferative disorder with risk to develop a systemic lymphoma".
  9. Magaña M, Sangüeza P, Gil-Beristain J, Sánchez-Sosa S, Salgado A, Ramón G; et al. (1998). "Angiocentric cutaneous T-cell lymphoma of childhood (hydroa-like lymphoma): a distinctive type of cutaneous T-cell lymphoma". J Am Acad Dermatol. 38 (4): 574–9. PMID 9580256.
  10. Kim TH, Lee JH, Kim YC, Lee SE (2014). "Hydroa vacciniforme-like lymphoma misdiagnosed as cutaneous lupus erythematosus". J Cutan Pathol. doi:10.1111/cup.12425. PMID 25488018.
  11. Wang T, Wang L, Xiong L, Han M, Liu W, Li G (2013). "A rare case of hydroa vacciniforme-like cutaneous T-cell lymphoma presenting mimicking herpes simplex in an adult". Am J Dermatopathol. 35 (5): 617–8. doi:10.1097/DAD.0b013e318277053c. PMID 23782639.
  12. 12.0 12.1 Quintanilla-Martinez L, Ridaura C, Nagl F, Sáez-de-Ocariz M, Durán-McKinster C, Ruiz-Maldonado R; et al. (2013). "Hydroa vacciniforme-like lymphoma: a chronic EBV+ lymphoproliferative disorder with risk to develop a systemic lymphoma". Blood. 122 (18): 3101–10. doi:10.1182/blood-2013-05-502203. PMID 23982171.
  13. Wang RC, Chang ST, Hsieh YC, Huang WT, Hsu JD, Tseng CE; et al. (2014). "Spectrum of Epstein-Barr virus-associated T-cell lymphoproliferative disorder in adolescents and young adults in Taiwan". Int J Clin Exp Pathol. 7 (5): 2430–7. PMC 4069894. PMID 24966953.
  14. "Hydroa-Like Cutaneous T-Cell Lymphoma: A Clinicopathologic and Molecular Genetic Study of 16 Pediatric Cases from Peru".
  15. Doeden K, Molina-Kirsch H, Perez E, Warnke R, Sundram U (2008). "Hydroa-like lymphoma with CD56 expression". J Cutan Pathol. 35 (5): 488–94. doi:10.1111/j.1600-0560.2007.00836.x. PMID 17976208.
  16. Boddu D, George R, Nair S, Bindra M, G Mathew L (2014). "Hydroa Vacciniforme-Like Lymphoma: A Case Report From India". J Pediatr Hematol Oncol. doi:10.1097/MPH.0000000000000221. PMID 25072366.
  17. Plaza JA, Sangueza M (2015). "Hydroa vacciniforme-like lymphoma with primarily periorbital swelling: 7 cases of an atypical clinical manifestation of this rare cutaneous T-cell lymphoma". Am J Dermatopathol. 37 (1): 20–5. doi:10.1097/DAD.0000000000000158. PMID 25162933.
  18. "Hydroa vacciniforme-like lymphoma: a chronic EBV+lymphoproliferative disorder with risk to develop a systemic lymphoma".
  19. 19.0 19.1 "Thalidomide for the treatment of hydroa vacciniforme-like lymphoma:Report of four pediatric cases from Peru".
  20. "Hydroa-Like Cutaneous T-Cell Lymphoma: A Clinicopathologic and Molecular Genetic Study of 16 Pediatric Cases from Peru".
  21. "Epstein–Barr Virus-Associated Hydroa Vacciniforme-like Cutaneous Lymphoma in Seven Chinese Children".
  22. Li HY, Wang HL, Gao TZ, Zhuo ZH, Li DM, Li HX (2009). "[Hydroa vacciniforme-like cutaneous T cell lymphoma: a case report and literature review]". Zhongguo Dang Dai Er Ke Za Zhi. 11 (7): 596–8. PMID 19651001.

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