Rieger E, Soyer H P, Leboit P E, Metze D, Slovak R, Kerl H
Department of Dermatology, University of Graz, Auenbruggerplatz 8, A-8036 Graz, Austria.
Br J Dermatol. 1999 Mar;140(3):497-504. doi: 10.1046/j.1365-2133.1999.02717.x.
Two elderly women with complex medical histories presented with erythematous patches, in one case involving the face and forearms, and in the other both elbows. Punch biopsies from both patients revealed intravascular proliferations of medium-sized and large cells with luminal occlusion typical of angioendotheliomatosis. Immunostaining did not show either lymphocytic or endothelial cell antigens but was consistent with a histiocytic differentiation of the intravascular cells in both cases, and was further substantiated by ultrastructural examination in one case. One patient received a course of cyclophosphamide therapy over 15 days. Skin lesions faded but did not disappear. The patient died 10 months later from cardiac and renal failure, which was most probably unrelated to the skin lesions. In the other case, lesions diminished but did not entirely resolve with treatment with low doses of oral prednisone. Angioendotheliomatosis can be divided into a malignant variant, which is an angiotropic lymphoma mostly of B-cell phenotype, and a benign, reactive variant, which is characterized by a proliferation of cells expressing endothelial cell markers. Only one case of angioendotheliomatosis with cells of histiocytic differentiation has been published previously under the name of intravascular histiocytosis. Our cases are very similar to the latter. The question arises as to whether intravascular histiocytic cell proliferation is a neoplastic proliferation of histiocytes or an early stage of classic reactive angioendotheliomatosis representing the residual cells associated with organization of microthrombi, which will be later followed by endothelial cell proliferation.
两名有着复杂病史的老年女性出现了红斑，一例累及面部和前臂，另一例累及双肘。两名患者的打孔活检均显示中、大细胞的血管内增生，伴有典型的血管内皮瘤病管腔闭塞。免疫染色未显示淋巴细胞或内皮细胞抗原，但在两例中均与血管内细胞的组织细胞分化一致，其中一例通过超微结构检查进一步证实。一名患者接受了为期15天的环磷酰胺治疗。皮肤病变消退但未消失。该患者10个月后死于心脏和肾衰竭，这很可能与皮肤病变无关。在另一例中，病变有所减轻，但低剂量口服泼尼松治疗后并未完全消退。血管内皮瘤病可分为恶性型，即主要为B细胞表型的嗜血管性淋巴瘤，以及良性反应型，其特征为表达内皮细胞标志物的细胞增殖。此前仅有一例组织细胞分化的血管内皮瘤病以血管内组织细胞增多症之名发表。我们的病例与后者非常相似。问题在于血管内组织细胞增殖是组织细胞的肿瘤性增殖，还是经典反应性血管内皮瘤病的早期阶段，代表与微血栓机化相关的残留细胞，随后会出现内皮细胞增殖。
