Michailidou Despina, Shin Junghee, Forde Inga, Gopalratnam Kavitha, Cohen Paul, DeGirolamo Angela
Department of Internal Medicine, Bridgeport Hospital, Yale-New Haven Health System, Bridgeport, CT, USA.
Section of Pulmonary, Critical Care and Sleep Medicine, Bridgeport Hospital, Yale-New Haven Health System, Bridgeport, CT, USA.
Auto Immun Highlights. 2015 Dec;6(3):39-46. doi: 10.1007/s13317-015-0071-9. Epub 2015 Sep 30.
Adult onset Still's disease (AOSD) is a systemic auto-inflammatory condition of unknown etiology, characterized by high fever, an evanescent, salmon-pink maculopapular skin rash, arthralgia or arthritis and leukocytosis. AOSD can also present with atypical cutaneous manifestations, such as persistent pruritic coalescent papules or plaques and linear lesions that have highly distinctive pathological features and are usually associated with severe disease. Herein, we present a 31-year-old Brazilian man with both typical Still's rash and atypical persistent polymorphic cutaneous manifestations associated with severe systemic inflammatory response syndrome. Eosinophils that are consistently lacking in the AOSD-associated skin lesions were evident in the skin biopsy of the persistent atypical cutaneous manifestations and were either drug-related or AOSD-associated.
成人斯蒂尔病（AOSD）是一种病因不明的全身性自身炎症性疾病，其特征为高热、一过性的鲑鱼粉红色斑丘疹、关节痛或关节炎以及白细胞增多。AOSD也可表现为非典型皮肤表现，如持续性瘙痒性融合丘疹或斑块以及具有高度独特病理特征且通常与严重疾病相关的线状损害。在此，我们报告一名31岁的巴西男性，其既有典型的斯蒂尔皮疹，又有与严重全身炎症反应综合征相关的非典型持续性多形性皮肤表现。在持续性非典型皮肤表现的皮肤活检中，可见在AOSD相关皮肤损害中一直缺乏的嗜酸性粒细胞，这些嗜酸性粒细胞与药物相关或与AOSD相关。
