Keller J, Spira I
Z Hautkr. 1986 Oct 1;61(19):1351-60.
We report on a rare case of Sweet's syndrome (SS) in a 7-year-old boy showing typical symptoms such as high fever, neutrophil leucocytosis in peripheral blood, elevated blood sedimentation rate, and arthralgia. The cutaneous lesions were painful erythematous, raised plaques on both limbs and the face. Histology confirmed the clinical diagnosis, including remarkable signs of cutaneous small vessel vasculitis, which was verified by direct immunofluorescence for IgM and complement (C3) in vessel walls as well as by the presence of circulating immune complexes in the blood. By means of monoclonal antibodies, T-lymphocytes and macrophages could be identified in the perivascular infiltrates in the delayed phase of SS. According to these immunological findings, we discuss the pathomechanism and the therapeutical benefit of inhibitors of cyclooxygenase.
我们报告了一例罕见的7岁男孩Sweet综合征（SS）病例，该男孩表现出典型症状，如高热、外周血中性粒细胞增多、血沉升高和关节痛。皮肤损害为四肢和面部疼痛性红斑、隆起斑块。组织学检查证实了临床诊断，包括皮肤小血管血管炎的显著征象，通过血管壁IgM和补体（C3）的直接免疫荧光以及血液中循环免疫复合物的存在得以验证。通过单克隆抗体，在SS延迟期的血管周围浸润中可识别出T淋巴细胞和巨噬细胞。根据这些免疫学发现，我们讨论了环氧化酶抑制剂的发病机制和治疗益处。
