以发热性溃疡坏死性急性痘疮样苔藓样糠疹为首发症状的间变性大细胞淋巴瘤1例

患儿女,7岁,以发热性溃疡坏死性急性痘疮样苔藓样糠疹为首发症状,半年后左下肢出现皮下肿物确诊为间变性大细胞淋巴瘤。患儿首发临床表现为全身皮肤水疱、溃疡、结痂伴发热、腹股沟淋巴结肿大。皮损组织病理:表皮可见角化不良细胞,界面改变,表皮少量淋巴细胞浸润,未见异型细胞;淋巴结病理:淋巴结大片坏死,其间血管壁坏死,周围绕以多数淋巴样细胞;肿物病理:大量淋巴样细胞弥漫分布。免疫组织化学:CD30(+),ALK胞浆(+),CD5(-),CD7(+),CD4(+),CD8(-),TIA-1(+),CD2(+),LCA(+),EMA(+),CD3(-),Vimentin(+),Ki-67(>80%+)。诊断:间变性大细胞淋巴瘤。

A Case of Large Cell Lymphoma with Febrile Ulceronecrotic Mucha-Habermann’s Disease as The First Symptom

A 7-year-old girl presented with febrile ulceronecrotic Mucha-Habermann’s disease as the first symptom.The first clinical manifestations of the child were systemic skin blisters,ulcers,scab with fever,inguinal lymph nodes enlargement.Skin histopathology showed:keratosis in the epidermis,changed interface,a small number of lymphocyte infiltration in the epidermis,no abnormal cells.Lymph node histopathology showed:large lymph nodes necrosis,vascular wall necrosis,surrounded by most lymphoid cells.Tumor histopathology showed:Diffuse distribution of many lymphoid cells.,CD30(+),ALK cytoplasm(+),CD5(-),CD7(+),CD4(+),CD8(-),TIA-1(+),CD2(+),LCA(+),EMA(+),CD3(-),Vimentin(+),Ki-67(>80%+).The diagnosis of anaplastic large cell lymphoma was made.