Perniciaro C, Winkelmann R K, Daoud M S, Su W P
Department of Dermatology, Mayo Clinic, Jacksonville, Florida 32224, USA.
Am J Dermatopathol. 1995 Jun;17(3):242-8. doi: 10.1097/00000372-199506000-00005.
Malignant angioendotheliomatosis is a rare intravascular (angiotropic) lymphoma. Patients most often present with cutaneous or central nervous system findings. We describe three patients with malignant angioendotheliomatosis involving the skin. The initial lesions in each were tender, indurated nodules on the lower extremities, resembling inflammatory panniculitis. Skin biopsies and immunohistochemical studies from all patients confirmed intravascular B-cell lymphoma. Two patients had visceral involvement, and molecular genetics studies showed clonal immunoglobulin gene rearrangement in one. Electron microscopy in this case showed increased fibrin and atypical lymphocytes within blood vessels. Malignant angioendotheliomatosis is a monoclonal intravascular lymphoma, usually of B-cell phenotype. Occlusion of small blood vessels with lymphoid cells, fibrin, and degenerating cellular debris causes the cutaneous lesions. An excisional biopsy through the depth of subcutaneous tissue may be necessary to confirm the diagnosis of malignant angioendotheliomatosis.
恶性血管内皮瘤病是一种罕见的血管内（亲血管性）淋巴瘤。患者最常表现出皮肤或中枢神经系统症状。我们描述了3例累及皮肤的恶性血管内皮瘤病患者。每位患者最初的皮损为下肢压痛性硬结性结节，类似炎性脂膜炎。所有患者的皮肤活检及免疫组化研究均证实为血管内B细胞淋巴瘤。2例患者有内脏受累，分子遗传学研究显示其中1例存在克隆性免疫球蛋白基因重排。该病例的电子显微镜检查显示血管内纤维蛋白增加及非典型淋巴细胞。恶性血管内皮瘤病是一种单克隆血管内淋巴瘤，通常为B细胞表型。小血管被淋巴细胞、纤维蛋白和退化的细胞碎片阻塞导致皮肤损害。可能需要通过皮下组织深度进行切除活检以确诊恶性血管内皮瘤病。
