种痘水疱病样皮肤T细胞淋巴瘤1例报道及文献复习

Hydroa vacciniforme-like cutaneous T cell lymphoma: a case report and literature review

摘要:

目的：探讨种痘水疱病样皮肤T细胞淋巴瘤的诊断与治疗，提高对该病的认识。方法：对1例种痘水疱病样皮肤T细胞淋巴瘤患儿的临床表现、辅助检查、诊疗过程进行分析，并复习相关文献。结果：该患儿主要表现为皮疹、发热、淋巴结肿大。皮疹呈多形性，表现为水肿性红斑、水疱、结痂、坏死及凹陷瘢痕，冬轻夏重，主要累及面部和四肢。EB（Epstein-Barr）病毒IgM抗体阳性。组织病理示皮肤真皮脂膜组织局灶性淋巴细胞浸润，以血管周围为主，免疫组化CD3 (+)、 CD43 (+)、 CD20 (-)、pax-5 (-)、TIA (+)、CD5 (+)、 CD8 (+)、 Granmye (+)、 CD4 (-)。激素治疗后病情缓解。结论：种痘水疱病样皮肤T细胞淋巴瘤的临床表现独特，确诊主要靠病损部位组织病理活检及免疫组化，部分病例对激素治疗反应良好，发病可能与EB病毒感染有关，预后有待于进一步观察。［中国当代儿科杂志，2009，11（7）：596-598］

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］