Valentín Sheila M, Montalván Elena, Sánchez Jorge L
Department of Dermatology, University of Puerto Rico, San Juan, Puerto Rico.
Am J Dermatopathol. 2010 Oct;32(7):716-9. doi: 10.1097/DAD.0b013e3181d775cf.
Hemophagocytosis may be encountered in association with a variety of underlying conditions, including primary or familial disorders, and secondary forms induced by infections, malignancy, and metabolic disorders. It is usually observed in organs such as the spleen, lymph nodes, bone marrow, and central nervous system, but rarely in the skin. When hemophagocytosis coexists with a sepsis-like systemic disorder it is termed hemophagocytic syndrome or hemophagocytic lymphohistiocytosis. Recently, 2 cases with evidence of perivascular hemophagocytosis in skin biopsy specimens of patients without additional findings of hemophagocytic lymphohistiocytosis have been reported. We report 2 additional cases of patients with cutaneous lesions suggestive of leukocytoclastic vasculitis whose skin biopsies showed a perivascular and interstitial infiltrate of neutrophils with nuclear dust and extravasated erythrocytes, and the presence of histiocytes with phagocytized red blood cells and nuclear fragments. There was also evidence of fibrin in the walls of the venules. One patient presented with a sepsis-like clinical scenario, but an extensive work-up failed to demonstrate any underlying disease or infection. The second patient was asymptomatic at the time of presentation, but further work-up revealed an underlying B-cell lymphoma. Whether these histologic findings represent late lesions of leukocytoclastic vasculitis or an incomplete presentation as part of a hemophagocytic syndrome is debatable, because both are associated with activated immunity and cytokine release, which could account for the presence of hemophagocytosis. Although the histopathologic finding alone of hemophagocytosis is insufficient to label as a syndrome, it should incite the clinician for further systemic evaluation.
噬血细胞现象可能与多种潜在疾病相关，包括原发性或家族性疾病，以及由感染、恶性肿瘤和代谢紊乱引起的继发性疾病。它通常在脾脏、淋巴结、骨髓和中枢神经系统等器官中观察到，但很少出现在皮肤中。当噬血细胞现象与类似败血症的全身性疾病共存时，称为噬血细胞综合征或噬血细胞性淋巴组织细胞增生症。最近，有报道称2例患者的皮肤活检标本中有血管周围噬血细胞现象的证据，但无噬血细胞性淋巴组织细胞增生症的其他表现。我们报告另外2例有提示白细胞破碎性血管炎的皮肤病变患者，其皮肤活检显示血管周围和间质有中性粒细胞浸润，伴有核尘和红细胞外渗，并有吞噬红细胞和核碎片的组织细胞。小静脉壁也有纤维蛋白的证据。1例患者表现出类似败血症的临床情况，但广泛检查未能发现任何潜在疾病或感染。第2例患者就诊时无症状，但进一步检查发现潜在的B细胞淋巴瘤。这些组织学发现是代表白细胞破碎性血管炎的晚期病变还是作为噬血细胞综合征的不完全表现存在争议，因为两者都与免疫激活和细胞因子释放有关，这可以解释噬血细胞现象的存在。虽然仅噬血细胞现象的组织病理学发现不足以诊断为综合征，但它应促使临床医生进行进一步的全身评估。
