Department of Dermatology and Dermatopathology, Royal Victoria Infirmary, Newcastle-Upon-Tyne, Tyne and Wear, UK.
Clin Exp Dermatol. 2011 Apr;36(3):288-91. doi: 10.1111/j.1365-2230.2010.03934.x. Epub 2010 Sep 16.
Intravascular lymphoma (IVL) is a subset of extranodal non-Hodgkin lymphoma, with an estimated incidence of <1 case per million people. It is characterised by extensive proliferation of lymphoma cells within small to medium-sized blood vessels. Most IVLs are B-cell tumours. IVL can present primarily in any organ system, including the skin. The disease is often disseminated at diagnosis. The overall mortality rate is thought to be >80%, and >50% of patients are diagnosed at postmortem examination. There is wide variability in the clinical appearance of cutaneous lesions, which may simulate inflammatory skin disease. Therefore, awareness by dermatologists is important to enable early diagnosis when cutaneous signs are present. We report two patients with unexplained systemic disease and a skin eruption, leading to the diagnosis of IVL, and outline the range of cutaneous features reported.
血管内淋巴瘤(IVL)是结外非霍奇金淋巴瘤的一个亚型,估计发病率为每百万人中有<1 例。其特征是小至中等大小血管内的淋巴瘤细胞广泛增殖。大多数 IVL 是 B 细胞肿瘤。IVL 可主要发生于任何器官系统,包括皮肤。该疾病在诊断时通常已广泛播散。总体死亡率被认为>80%,>50%的患者是在尸检时诊断出来的。皮肤病变的临床表现差异很大,可能模拟炎症性皮肤病。因此,皮肤科医生的意识很重要,以便在出现皮肤征象时能够早期诊断。我们报告了两名患有不明原因的系统性疾病和皮疹的患者,导致 IVL 的诊断,并概述了报告的一系列皮肤特征。