Aberer W, Konrad K, Wolff K
Department of Dermatology I, University of Vienna, Austria.
J Am Acad Dermatol. 1988 Jan;18(1 Pt 1):105-14. doi: 10.1016/s0190-9622(88)70016-x.
Wells' syndrome is a distinctive dermatosis clinically resembling acute cellulitis with solid edema; it resolves spontaneously after weeks or months without residues. Recurrences over many years are common. Light microscopy is characteristic for the disease, with diffuse tissue eosinophilia and marked edema, fibrinoid "flame figures," and palisading microgranuloma. Vasculitis is never found. Eosinophilia of the peripheral blood is a frequent feature. Etiology and pathogenesis are unknown, but the disease has been found to be associated with hematologic disorders in several cases, and recurrences can often be related to infections, arthropod bites, drug administration, or surgery. The diagnosis of Wells' syndrome should be based on the typical clinical picture and the course of the disease with its recurrences and histopathology. Flame figures in histologic sections are an important diagnostic feature but not diagnostic per se for the disease because they represent a reaction pattern that can occur in other conditions. A dilution of Wells' syndrome by making flame figures the central criterion of diagnosis and by lumping all flame figure-positive skin reactions together is therefore unjustified.
威尔斯综合征是一种独特的皮肤病,临床上类似于伴有实性水肿的急性蜂窝织炎;数周或数月后可自行消退,不留痕迹。多年复发很常见。光镜检查是该疾病的特征性表现,有弥漫性组织嗜酸性粒细胞增多、明显水肿、纤维蛋白样“火焰状图形”和栅栏状微肉芽肿。从未发现血管炎。外周血嗜酸性粒细胞增多是常见特征。病因和发病机制尚不清楚,但在几例病例中发现该疾病与血液系统疾病有关,复发通常与感染、节肢动物叮咬、药物给药或手术有关。威尔斯综合征的诊断应基于典型的临床表现、疾病的病程及其复发情况以及组织病理学。组织学切片中的火焰状图形是一个重要的诊断特征,但本身并非该疾病的诊断依据,因为它们代表了一种可在其他情况下出现的反应模式。因此,将火焰状图形作为诊断的核心标准并将所有火焰状图形阳性的皮肤反应归为一类来淡化威尔斯综合征的诊断是不合理的。