中国麻风皮肤病杂志 ›› 2023, Vol. 39 ›› Issue (9): 647-652.doi: 10.12144/zgmfskin202309647

• 论著 • 上一篇    下一篇

种痘样水疱病样淋巴组织增生性疾病伴乳腺转移一例并文献复习

罗志强,吴玮,谢锦莹,李梅赵,周书文,汪凤娟,周海园   

  1. 广东医科大学附属医院皮肤科,广东湛江,524000
  • 出版日期:2023-09-15 发布日期:2023-09-05

Hydroa vacciniforme-like lymphoproliferative disorder with breast metastasis: a case report and literature review

LUO Zhiqiang, WU Wei, XIE Jinying, LI Meizhao, ZHOU Shuwen, WANG Fengjuan, ZHOU Haiyuan   

  1. Department of Dermatology, Affiliated Hospital of Guangdong Medical University, Zhanjiang 524000, China
  • Online:2023-09-15 Published:2023-09-05

摘要: 种痘样水疱病样淋巴组织增生性疾病(HVLPD)可累及皮肤、肝脾及骨髓等器官,但累及乳腺尚未见报道。本文报道一例并复习相关文献。患者,女,19岁。全身反复红斑、血疱伴高热17年,右面部肿胀4个月。皮损组织病理示:真皮全层,尤其是血管、附属器周围以及脂肪小叶内见大量淋巴样细胞浸润,部分呈浆细胞样,细胞轻度异型,见异常核分裂像。免疫组化示:CD3、CD4、CD8、CD20、CD45RO均强阳性;EBER(+)。乳腺肿物穿刺病理活检:部分区域乳腺小叶内见较多淋巴细胞浸润,EBER(+)。诊断:种痘样水疱病样淋巴组织增生性疾病伴乳腺转移。患者辗转在乳腺外科、风湿科、烧伤外科治疗后小腿溃疡扩大伴出血,再次左小腿溃疡行组织病理示种痘样水疱样EB病毒相关淋巴组织增殖性疾病伴淋巴瘤转化(结外NK/T细胞淋巴瘤),发病19年后患者死亡。

关键词: 种痘样水疱病样淋巴组织增生性疾病, EB病毒, 乳腺

Abstract: Hydroa vacciniforme-like lymphoproliferative disorder (HVLPD) can involve organs such as skin, liver , spleen, and bone marrow, but involvement of the breast has not been reported. Herein, a case is reported and relevant literature reviewed. A 19-year-old female patient presented with recurrent erythema, blood blisters and high fever for 17 years, and right facial swelling for 4 months.  Histopathology of the skin lesion showed a large number of lymphoid cells were infiltrated in the whole dermis, especially around blood vessels, appendages and fat lobules, some of which were plasmacytoid, with mild cell atypia and abnormal mitosis.  Immunohistochemistory showed CD3, CD4,CD8, CD20 and CD45RO were strongly positive. EBER(+). Puncture pathological biopsy of breast tumor: more lymphocyte infiltration was observed in lobules in some areas, EBER (+).  The final diagnosis was hydroa vacciniforme-like lymphoproliferative disorder with breast metastasis. After treatment in the breast surgery department, rheumatology department, and burn surgery department, the patient had an enlarged leg ulcer accompanied by bleeding. And the histopathology of the left leg ulcer showed that transformation of lymphoproliferative disease associated with varicella vesicular disease-like EB virus infection to lymphoma  (extranodal NK/T-cell lymphoma). The patient finally died 19 years after the disease.

Key words: hydroa vacciniforme-like lymphoproliferative disorder, Epstein-Barr virus, breast