Noyon G, Blanc D, Kienzler J L, Laurent R, Estavoyer J M, Dupont J L
Ann Dermatol Venereol. 1983;110(2):107-11.
The authors report four new cases of Still's disease in adult. Each time, the clinical picture associated the following triad: fever, joint involvement and skin rashes. The latter consisted in non-pruritic but sensitive maculo-papular and disseminated lesions, the mean course of which lasted 24 to 48 hours and were readily accompanied by the two other components of the basic clinical triad. Laboratory investigations were non-specific but constantly included a polymorphonuclear leucocytosis and a raised ESR, the mean value of which reached 80 mm in the 1st hour. Antinuclear factors were not demonstrated, but circulating immune complexes were found on several occasions in three out of the four reported cases. Histopathological changes observed were those of a superficial and mid cutis edema, associated with a diffuse or perivascular infiltrate in which either mononuclears or polymorphonuclears were prominent. Dermal vessels involvement appeared rather weak, including swelling of the walls by edema with focal fibrinoid necrosis. Direct immunofluorescence showed mainly IgG, IgA and C3 vascular deposits. Based on these data, an immune pathology is discussed, besides the more classic infections pathogenesis.
作者报告了4例成人斯蒂尔病新病例。每次，临床表现均包含以下三联征：发热、关节受累和皮疹。皮疹表现为非瘙痒性但有触痛的斑丘疹和散在性损害，其平均病程持续24至48小时，并常伴有基本临床三联征的其他两个组成部分。实验室检查无特异性，但始终包括多形核白细胞增多和血沉升高，第1小时血沉平均值达80mm。未发现抗核因子，但在4例报告病例中的3例中多次发现循环免疫复合物。观察到的组织病理学变化为浅表和真皮中部水肿，伴有弥漫性或血管周围浸润，其中单核细胞或多形核白细胞突出。真皮血管受累似乎较轻，包括血管壁因水肿而肿胀并伴有局灶性纤维蛋白样坏死。直接免疫荧光主要显示IgG、IgA和C3血管沉积。基于这些数据，除了更经典的感染发病机制外，还讨论了免疫病理学。
