毛细血管扩张样表现的皮肤血管内大B细胞淋巴瘤1例

Cutaneous Intravascular Large B-Cell Lymphoma Presenting with Telangiectasia:A Case Report

患者男,58岁,双下肢水肿性痛性红斑伴间断发热2个月,同时出现发作性谵妄、意识模糊。皮肤科情况:胸腹部及双下肢皮肤见多处蜘蛛痣样毛细血管扩张,压之褪色。局部皮肤呈橘皮样外观,有浸润感,触痛。双下肢近端皮肤见紫红色斑。双下肢凹陷性水肿。皮损组织病理示:真皮及皮下脂肪组织小血管内见呈巢团分布的异型淋巴样细胞,细胞形态单一,充满整个血管腔。免疫组织化学:CD20(+),CD5(+),Bcl-2(80%+),c-Myc(10%+),MUM1(+),Ki-67(80%+)。基因重排结果:IgK-A(+),IgL(+)。诊断:皮肤血管内大B细胞淋巴瘤。

A 58-year-old male presented with edematous and painful erythematous plaques at both lower extremities,accompanied by intermittent fever for two months.Moreover,the patient also presented with delirium and confusion.Physical examination showed some spider-like telangiectasis on the chest,abdomen and lower extremities,which faded under pressure.The local skin had an orange peel-like appearance with inflammation and tenderness.A purple-red spot was seen on proximal limbs.There was pitting edema in both lower limbs.Histopathology showed nests of heteromorphic lymphoid cells in the small vessels of the dermis and subcutaneous adipose tissue.The single cells filled the whole blood vessel cavity.Immunohistochemistry showed positive stains for CD20,CD5,MUM1,Bcl-2(about 80%),c-Myc(about 10%)and Ki-67(about 80%).Gene rearrangement was IgK-A and IgL positive.Diagnosed:cutaneous intravascular large B-cell lymphoma.