Ito K, Hara H, Okada T, Terui T
Department of Dermatology, Kawaguchi Medical Center, Saitama, Japan.
Clin Exp Dermatol. 2009 Jul;34(5):e192-5. doi: 10.1111/j.1365-2230.2008.03008.x. Epub 2008 Dec 9.
Hypereosinophilic syndrome (HES) is a multisystem disease with a high mortality rate. It is characterized by peripheral blood eosinophilia and eosinophilic infiltration of the skin and many other organs. The commonest cutaneous features include erythematous pruritic maculopapules and nodules, angio-oedema or urticarial plaques. However, some case reports have indicated that eosinophilic cellulitis, cutaneous necrotizing eosinophilic vasculitis, Raynaud's phenomenon and digital gangrene may also occur as cutaneous features of HES. Juvenile temporal arteritis (JTA) of unknown cause is characterized by an asymptomatic nodule in the temporal artery area in young adults. Histologically, the lesion is characterized by a significant intimal thickening with moderate eosinophilic infiltrates, constriction or occlusion of the vascular lumen and absence of giant cells. We report a patient with HES presenting with eosinophilic cellulitis, Raynaud's phenomenon, digital gangrene and JTA. JTA may also be one of the features of HES.
高嗜酸性粒细胞综合征(HES)是一种死亡率很高的多系统疾病。其特征为外周血嗜酸性粒细胞增多以及皮肤和许多其他器官的嗜酸性粒细胞浸润。最常见的皮肤表现包括红斑性瘙痒性斑丘疹和结节、血管性水肿或荨麻疹斑块。然而,一些病例报告表明,嗜酸性蜂窝织炎、皮肤坏死性嗜酸性血管炎、雷诺现象和指端坏疽也可能作为HES的皮肤表现出现。病因不明的青少年颞动脉炎(JTA)的特征是在年轻成年人的颞动脉区域出现无症状结节。组织学上,病变的特征是显著的内膜增厚伴中度嗜酸性粒细胞浸润、血管腔狭窄或闭塞且无巨细胞。我们报告一例患有HES的患者,其表现为嗜酸性蜂窝织炎、雷诺现象、指端坏疽和JTA。JTA也可能是HES的特征之一。