Abstract OBJECTIVE: To study the clinical features, diagnosis and therapy of hydroa vacciniforme-like cutaneous T cell lymphoma. METHODS: The clinical presentations and the findings of laboratory examinations and skin biopsy of affected tissue in a child with hydroa vacciniforme-like cutaneous T cell lymphoma were retrospectively reviewed. RESULTS: The child manifested as rash, fever and lymph node intumesce. Rash was pantomorphia, including edematous erythema, vesicles, crusts, necrosis and depressed scar, and it was mild in winter and severe in summer, mainly involving in the face and extremities. Epstein-Barre vivus (EBV)-IgM was positive. Histopathological findings revealed focal lymphocyte invasion in subcutaneous panniculus adiposus, mainly surrounding the blood vessels. Immunohistochemistory showed CD3 (+), CD43 (+), CD20 (-), pax-5 (-), TIA (+), CD5 (+), CD8 (+), Granmye (+) and CD4 (-). The clinical symptoms were improved after glucocorticoid treatment in this child. CONCLUSIONS: Hydroa vacciniforme-like cutaneous T cell lymphoma has special clinical manifestations. This disorder may be definitely diagnosed by skin biopsy of affected tissue and immunohistochemistry assay. Glucocorticoid treatment is effective. EBV infection may be related to the development of this disorder.［Chin J Contemp Pediatr, 2009, 11 (7):596-598］
LI Hai-Ying,WANG Huai-Li,GAO Tie-Zheng et al. Hydroa vacciniforme-like cutaneous T cell lymphoma: a case report and literature review[J]. 中国当代儿科杂志, 2009, 11(07): 596-598.
LI Hai-Ying,WANG Huai-Li,GAO Tie-Zheng et al. Hydroa vacciniforme-like cutaneous T cell lymphoma: a case report and literature review[J]. CJCP, 2009, 11(07): 596-598.
［1］Barrionuevo C, Anderson VM, Zevallos-Giampietri E, Zaharia M, Misad O, Bravo F, et al. Hydroa-like cutaneous T-cell lymphoma:a clinicopathologic and molecular genetic study of 16 pediatric cases from Peru［J］. Appl Immunohistochem Mol Morphol, 2002, 10(1):7-14.
［2］Magana M, Sangueza P, Gil-Beristain J, Sanchez-Sosa S, Salgado A, Ramon G, et al. Angiocentric cutaneous T-cell lymphoma of childhood (hydroa-like lymphoma):a distinctive type of cutaneous T-cell lymphoma［J］. J Am Acad Dermatol, 1998, 38(4):574-579.
［4］Yukiko N, Keiji I, Hiroshi K, Seiji K, Tsuneo M, Kazuhide T, et al. Fatal natural killer cell lymphoma arising in a patient with a crop of Epstein-Barr virus-associated disorder［J］. Eur J Dermatol, 2005, 15(6):503-506.
［8］Iwatsuki K, Xu Z, Ohtsuka M, Kaneko F. Cutaneous lymphoproliferative disorders associated with Epstein-Barr virus infection:a clinical overview［J］. J Dermatol Sci, 2000, 22(3):181-195.
［9］Okamura T, Kishimoto T, Inoue M, Honda M, Yamashita N, Wakiguchi H, et al. Unrelated bone marrow transplantation for Epstein-Barr virusassociated T/NK-cell lymphoproliferative disease［J］. Bone Marrow Transplant, 2003, 31(2):105-111.
