Lee Jee Bum, Kim Jae Wook, Lee Shin Seok, Kim Seong Jin, Won Young Ho, Lee Seung Chul
Department of Dermatology, Chonnam National University Medical School, Gwangju, Korea.
J Korean Med Sci. 2002 Dec;17(6):852-5. doi: 10.3346/jkms.2002.17.6.852.
Adult-onset Still's disease (AOSD) is a rare systemic inflammatory disorder of unknown etiology characterized by an evanescent rash, intermittent spiking high fever, arthralgia, and a variety of systemic features. We describe a 46-yr-old woman with Still s disease who presented with a 2-month history of a symmetrical vesiculopustular eruptions only on both hands and feet. Skin biopsy specimens of both vesicle and pustule revealed fibrin thrombi deposition in the small dermal vessels with little inflammation, subepidermal bulla, and ischemic necrosis of the overlying epidermis, which were consistent with a vasculopathic reaction. Her skin lesions gradually disappeared in response to conventional AOSD therapy, as elevated serum ferritin levels, an index of disease activity, decreased. To the best of our knowledge, this is the first case of vesiculopustular lesions as a skin manifestation of AOSD.
成人斯蒂尔病(AOSD)是一种病因不明的罕见全身性炎症性疾病,其特征为一过性皮疹、间歇性高热、关节痛以及多种全身症状。我们描述了一名46岁患有斯蒂尔病的女性,她仅双手和双脚出现对称性水疱脓疱疹2个月。水疱和脓疱的皮肤活检标本显示真皮小血管中有纤维蛋白血栓沉积,炎症轻微,表皮下大疱形成,上方表皮缺血坏死,这些表现符合血管病变反应。随着疾病活动指标血清铁蛋白水平升高的情况有所下降,她的皮肤病变在接受传统的AOSD治疗后逐渐消失。据我们所知,这是首例以水疱脓疱疹作为AOSD皮肤表现的病例。