Chen K R, Su W P, Pittelkow M R, Leiferman K M
Department of Dermatology, Mayo Clinic, Rochester, MN 55905, USA.
Semin Dermatol. 1995 Jun;14(2):106-10. doi: 10.1016/s1085-5629(05)80005-7.
We recently identified a syndrome of recurrent cutaneous eosinophilic vasculitis in three patients. These patients had in common widespread pruritic, erythematous, purpuric papules and angioedema of face and hands associated with peripheral blood eosinophilia. Eight skin biopsies from these three patients all showed necrotizing vasculitis of the small vessels of the skin, with exclusively eosinophilic infiltration and minimal or no leukocytoclasis. The disease followed a chronic course, with recurrent, itchy, swelling skin lesions and without evidence of systemic involvement over observation periods of 3, 17, and 23 years. The skin lesions responded promptly to systemic steroid treatment, but two patients required maintenance doses for control of the disease. Immunofluorescence studies showed marked deposition of the cytotoxic eosinophil granule major basic protein in the affected vessel walls. Eosinophil-active cytokine IL-5 was detected in the serum of one patient. Expression of the vascular cell adhesion molecule-1 for eosinophil adherence was detected on the endothelium of the affected vessels. Because this disease showed distinctive clinical manifestations and characteristic histopathological features, we believe it is a distinct entity and should be distinguished from other types of vasculitis.
我们最近在三名患者中发现了一种复发性皮肤嗜酸性血管炎综合征。这些患者的共同特点是广泛存在瘙痒性、红斑性、紫癜性丘疹以及面部和手部血管性水肿，并伴有外周血嗜酸性粒细胞增多。这三名患者的八次皮肤活检均显示皮肤小血管坏死性血管炎，仅有嗜酸性粒细胞浸润，白细胞破碎现象轻微或无。该病呈慢性病程，皮肤病变反复出现、瘙痒、肿胀，在3年、17年和23年的观察期内均无全身受累迹象。皮肤病变对全身类固醇治疗反应迅速，但两名患者需要维持剂量来控制病情。免疫荧光研究显示，在受累血管壁中有明显的细胞毒性嗜酸性粒细胞颗粒主要碱性蛋白沉积。在一名患者的血清中检测到嗜酸性粒细胞活性细胞因子IL-5。在受累血管的内皮细胞上检测到了用于嗜酸性粒细胞黏附的血管细胞黏附分子-1的表达。由于这种疾病表现出独特的临床表现和特征性组织病理学特征，我们认为它是一种独特的疾病实体，应与其他类型的血管炎相区分。
