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Vol. 226, No. 3, 2013
Issue release date: August 2013
Dermatology 2013;226:212-216

Chronic Active Epstein-Barr Virus Infection-Associated Hydroa Vacciniforme-Like Eruption and Behçet's-Like Orogenital Ulcers

Park B.M. · Ahn J.-S. · Lee J.B. · Won Y.H. · Yun S.J.
Departments of aDermatology and bHematology-Oncology, Chonnam National University Medical School, Gwangju, South Korea

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The cutaneous manifestations of chronic active Epstein-Barr virus (EBV) infection can be diverse. Among them, hydroa vacciniforme-like eruption is one of the best-known features. Although rare, mucosal ulcers have been reported to be associated with EBV as a result of primary infection or immune suppression. We describe a 65-year-old female with recurrent necrotic papulovesicles on the face and both arms for 2 years. She also complained of recurrent oral and genital mucosal ulcers developing simultaneously with skin eruptions. They appeared periodically during the spring and summer and were triggered or aggravated by sun exposure. Skin biopsies from the face and genitalia showed identical findings with dense lymphocytic infiltrations. In addition, in situ hybridization revealed EBV-positive lymphoid cells in both specimens. To our knowledge, this is the first case of serologically and pathologically proven chronic active EBV infection presenting hydroa vacciniforme-like eruption and orogenital ulcers at the same time in one patient.

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  1. Cohen JI: Epstein-Barr virus infection. N Engl J Med 2000;343:481-492.
  2. Kimura H: Pathogenesis of chronic active Epstein-Barr virus infection: is this an infectious disease, lymphoproliferative disorder, or immunodeficiency? Rev Med Virol 2006;16:251-261.
  3. Iwatsuki K, Xu Z, Ohtsuka M, Kaneko F: Cutaneous lymphoproliferative disorders associated with Epstein-Barr virus infection: a clinical overview. J Dermatol Sci 2000;22:181-195.
  4. Iwatsuki K, Ohtsuka M, Harada H, Han G, Kaneko F: Clinicopathologic manifestations of Epstein-Barr virus-associated cutaneous lymphoproliferative disorders. Arch Dermatol 1997;133:1081-1086.
  5. Al-Rawahi GN, Dobson SR, Scheifele DW, Rassekh SR, Murphy JJ: Severe genital ulceration in an acute Epstein-Barr virus infection. Pediatr Infect Dis J 2011;30:176-178.
  6. Barnes CJ, Alio AB, Cunningham BB, Friedlander SF: Epstein-Barr virus-associated genital ulcers: an under-recognized disorder. Pediatr Dermatol 2007;24:130-134.
  7. Cheng SX, Chapman MS, Margesson LJ, Birenbaum D: Genital ulcers caused by Epstein-Barr virus. J Am Acad Dermatol 2004;51:824-826.
  8. Halvorsen JA, Breviq T, Aas T, Skar AG, Slevolden EM, Moi H: Genital ulcers as initial manifestation of Epstein-Barr virus infection: two new cases and a review of the literature. Acta Derm Venereol 2006;86:439-442.
  9. Lampert A, Assier-Bonnet H, Chevallier B, Clerici T, Saiag P: Lipschutz's genital ulceration: a manifestation of Epstein-Barr virus primary infection. Br J Dermatol 1996;135:663-665.
  10. Sardy M, Wollenberg A, Niedermeier A, Flaig MJ: Genital ulcers associated with Epstein-Barr virus infection (ulcus vulvae acutum). Acta Derm Venereol 2011;91:55-59.

    External Resources

  11. Dojcinov SD, Venkataraman G, Raffeld M, Pittaluga S, Jaffe ES: EBV positive mucocutaneous ulcer - a study of 26 cases associated with various sources of immunosuppression. Am J Surg Pathol 2010;34:405-417.
  12. McGinness JL, Spicknall KE, Mutasim DF: Azathioprine-induced EBV-positive mucocutaneous ulcer. J Cutan Pathol 2012;39:377-381.
  13. Cho KH, Lee SH, Kim CW, Jeon YK, Kwon IH, Cho YJ, Lee SK, Suh DH, Chung JH, Yoon TY, Lee SJ: Epstein-Barr virus-associated lymphoproliferative lesions presenting as a hydroa vacciniforme-like eruption: an analysis of six cases. Br J Dermatol 2004;151:372-380.
  14. Xu Z, Lian S: Epstein-Barr virus-associated hydroa vacciniforme-like cutaneous lymphoma in seven Chinese children. Pediatr Dermatol 2010;27:463-469.
  15. Wu YH, Chen HC, Hsiao PF, Tu MI, Lin YC, Wang TY: Hydroa vacciniforme-like Epstein-Barr virus-associated monoclonal T-lymphoproliferative disorder in a child. Int J Dermatol 2007;46:1081-1086.
  16. Cho KH, Kim CW, Heo DS, Lee DS, Choi WW, Rim JH, Han WS: Epstein-Barr virus-associated peripheral T-cell lymphoma in adults with hydroa vacciniforme-like lesions. Clin Exp Dermatol 2001;26:242-247.
  17. Cho KH, Kim CW, Lee DY, Sohn SJ, Kim DW, Chung JH: An Epstein-Barr virus-associated lymphoproliferative lesion of the skin presenting as recurrent necrotic papulovesicles of the face. Br J Dermatol 1996;134:791-796.
  18. Ruiz-Maldonado R, Parrilla FM, Orozco-Covarrubias ML, Ridaura C, Tamayo Sanchez L, Duran McKinster C: Edematous, scarring vasculitic panniculitis: a new multisystemic disease with malignant potential. J Am Acad Dermatol 1995;32:37-44.
  19. Magana M, Sangueza P, Gil-Beristain J, et al: Angiocentric cutaneous T-cell lymphoma of childhood (hydroa-like lymphoma): a distinctive type of cutaneous T-cell lymphoma. J Am Acad Dermatol 1998;38:574-579.
  20. Iwatsuki K, Xu Z, Takata M, et al: The association of latent Epstein-Barr virus infection with hydroa vacciniforme. Br J Dermatol 1999;140:715-721.
  21. Nishizawa A, Satoh T, Takayama K, Yokozeki H: Hydroa vacciniforme with mucosal involvement and recalcitrant periodontitis and multiple virus re-activators after sun-exposure. Acta Derm Venereol 2010;90:498-501.
  22. Sunohara A, Mizuno N, Sakai M, Kawabe Y, Sakakibara S: Action spectrum for UV erythema and reproduction of the skin lesions in hydroa vacciniforme. Photodermatology 1988;5:139-145.

    External Resources

  23. Heo EP, Park SH, Kim TH: Artificial reproduction of atypical hydroa vacciniforme caused by latent Epstein-Barr virus infection. Int J Dermatol 2003;42:476-479.
  24. Schwarz T: Photoimmunosuppression. Photodermatol Photoimmunol Photomed 2002;18:141-145.
  25. Sun A, Chang JG, Chu CT, Liu BY, Yuan JH, Chiang CP: Preliminary evidence for an association of Epstein-Barr virus with pre-ulcerative oral lesions in patients with recurrent aphthous ulcers or Behçet's disease. J Oral Pathol Med 1998;27:168-175.
  26. Hooks JJ: Possibility of a viral etiology in recurrent aphthous ulcers and Behçet's syndrome. J Oral Pathol 1978;7:353-364.

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