Carlesimo M, Fidanza L, Mari E, Feliziani G, Narcisi A, De Marco G, Bartolazzi A, Camplone G
U.O.C. Dermatology, II Unit University of Rome 'Sapienza', Rome, Italy.
Case Rep Dermatol. 2009 Sep 12;1(1):44-48. doi: 10.1159/000236147.
Eosinophil-associated diseases represent a spectrum of heterogeneous disorders, where blood and cutaneous eosinophilia is the most important feature and eosinophils are the principal cause of cutaneous lesions. These diseases show some similarities in the clinical features but also many distinctive characteristics [Saurat et al., Dermatologia e malattie sessualmente trasmesse, Milano, Masson, 2000]. Wells syndrome is one of these disorders and is an uncommon recurrent inflammatory dermatosis, rarely associated to signs and symptoms of multiple organ involvement [Arch Dermatol 2006;142:1157-1161]. Hypereosinophilic syndrome, in contrast, constitutes a group of idiopathic disorders characterized by blood eosinophilia for at least 6 months, associated with single or multiple organ system dysfunction [Arch Dermatol 2006;142:1157-1161]. Clinically atypical Wells syndrome with multiorgan involvement is reported here. A correct diagnosis is difficult in this case, but clinical and histopathological features are compatible with this diagnosis. The reported condition likely represents a borderline hypereosinophilic disease, in which clinical features of both hypereosinophilic syndrome and Wells syndrome are present.
嗜酸性粒细胞相关疾病是一系列异质性疾病，其中血液和皮肤嗜酸性粒细胞增多是最重要的特征，且嗜酸性粒细胞是皮肤病变的主要原因。这些疾病在临床特征上有一些相似之处，但也有许多独特的特点[索拉特等人，《性传播疾病与皮肤病学》，米兰，马松出版社，2000年]。韦尔斯综合征就是其中之一，是一种罕见的复发性炎症性皮肤病，很少伴有多器官受累的体征和症状[《皮肤病学文献》2006年；142:1157 - 1161]。相比之下，高嗜酸性粒细胞综合征是一组特发性疾病，其特征为血液嗜酸性粒细胞增多至少6个月，并伴有单器官或多器官系统功能障碍[《皮肤病学文献》2006年；142:1157 - 1161]。本文报告了伴有多器官受累的临床非典型韦尔斯综合征。在此病例中做出正确诊断很困难，但临床和组织病理学特征与该诊断相符。所报告的病情可能代表一种边缘性高嗜酸性粒细胞疾病，其中同时存在高嗜酸性粒细胞综合征和韦尔斯综合征的临床特征。
