Typical evanescent and atypical persistent polymorphic cutaneous rash in an adult Brazilian with Still's disease: a case report and review of the literature.
Typical evanescent and atypical persistent polymorphic cutaneous rash in an adult Brazilian with Still's disease: a case report and review of the literature.
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DOI：
10.1007/s13317-015-0071-9
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发表时间：
2015-12
期刊：
影响因子：
--
通讯作者：
DeGirolamo A
中科院分区：
文献类型：
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作者：
Michailidou D;Shin J;Forde I;Gopalratnam K;Cohen P;DeGirolamo A
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，其特征是高热、一过性橙红色斑丘疹、关节痛或关节炎和白细胞增多。AOSD也可表现为非典型的皮肤表现，如持续性皮炎合并性丘疹或斑块和线性病变，具有高度独特的病理特征，通常与严重疾病相关。在此，我们提出了一个31岁的巴西男子与典型的斯蒂尔皮疹和非典型的持续多形性皮肤表现与严重的全身炎症反应综合征。在持续性非典型皮肤表现的皮肤活检中，在AOSD相关皮肤病变中始终缺乏嗜酸性粒细胞，并且与药物相关或与AOSD相关。
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.