儿童金黄色葡萄球菌烫伤样皮肤综合征课件

儿童金黄色葡萄球菌烫伤样皮肤综合征,湘西州人民医院儿二科 危坚,Staphylococcal Scalded Skin Syndrome,the Ritters disease,概述,Staphylococcus aureus causes a variety of infectious diseases，ranging from superficial skin infections to severe,toxin-mediated systemic infections. 金黄色葡萄球菌可引起各种各样的感染性疾病，包括严重的表皮感染和毒素介导的全身性感染。 S. aureus produces many extracellular products, including toxins, that affect host cell function or morphology. Staphylococcal toxic shock syndrome (STSS) and staphylococcal scalded skin syndrome (SSSS) are 2 distinct toxin-mediated diseases with very distinct cutaneous features. 金黄色葡萄球菌产生大量细胞外产物，包括毒素，影响宿主细胞功能和形态。葡萄球菌中毒性休克综合征（STSS）和金黄色葡萄球菌烫伤样皮肤综合征（SSSS）是两种独特的以皮肤受累为特征的毒素介导的疾病。,概述,金黄色葡萄球菌性烫伤样皮肤综合征是一种急性感染性皮肤病，临床特征为全身泛发性红斑、松弛性大疱、表皮剥脱及尼氏征阳性，重症者可并发败血症，发生多器官系统功能受累，甚至危及生命。 该病好发于婴幼儿，可能由于6岁以下的儿童特异性免疫尚未发育完善，体内抗表皮剥脱毒素的抗体不足，肾脏排泄毒素的能力较差。,流行病学,葡萄球菌性烫伤样皮肤综合征（staphylococcal scalded skin syndrome，SSSS），是一种主要由凝固酶阳性嗜菌体组71型和51型金黄色葡萄球菌引起。 Ladhani等于1999年报道本病病死率3%-4%。 好发于6岁及以下幼儿，罕见的成人葡萄球菌性烫伤样皮肤综合征患者伴有原发疾病，如免疫抑制、免疫异常、肾功能不全等。 一般病程在12周。,蔡爱东，李军，周兆群，等. 儿童葡萄球菌烫伤样皮肤综合征36例临床析J. 实用医学杂志，2010，2（14）:2594-2595.,临床表现,病程早期自口、眼四周等部位出现皮肤损害，主要为红斑、水疱，随着病程的进展，皮疹范围迅速扩大，蔓延至颈、胸、背、腋下、腹股沟等部位，表现为弥漫性红斑及红斑基础上松弛性水疱、大疱，水疱破裂后表皮剥脱，类似烫伤样，尼氏征阳性。 患儿感明显的疼痛，不能触碰。 首发症状：65例首发临床表现中口周放射状皲裂，眶周红斑、睑缘糜烂并伴有脓性分泌物。,儿童金黄色葡萄球菌性烫伤样皮肤综合征（SSSS）168例临床 及实验室检测分析,China Continuing Medical Education, Vol. 7, No.24,临床表现,患儿均为全身型，发病时皮疹最先见于面部，尤其是口、眼四周的无特异性皮肤红斑，后迅速向下蔓延到腋、腹股沟、躯干和四肢近端，直至泛发到全身。 2-3d内迅速蔓延全身广泛分布，在弥漫性红斑上出现松弛大疱，其上表皮皱起，尼氏征阳性。表皮易剥脱而露出鲜红色水肿糜烂面，特征类似于度烫伤样皮损。 1-2d后可见痂皮脱屑，口周呈特征性的放射状皲裂，手足皮肤可呈手套或袜套样脱皮，以后不再剥脱，而出现糠秕状脱屑。,Berk，David R，Bayliss，et al. MRSA，Staphylococcal Scalded Skin Syndrome，and Other Cutaneous Bacterial EmergenciesJ. Pediatric Annals，2010，10（39）:627-633.,临床表现,Desquamation and erosions are seen on the trunk. 躯干可见皮肤脱屑和侵蚀,A six-year-old boy was hospitalized with a rash and fever.,一个6岁男孩因发热和皮疹入院。,患儿入院2天前出现皮肤瘙痒。入院当晚，患儿鼻子和面部出现小水疱。在随后的2天，尽管使用头孢氨苄治疗，皮损区域扩散。他的面部，脖子，后背部，肛周，大腿，腋窝大量松弛的大水疱样皮疹。尼氏征阳性（上皮表层从基底层分离）。皮肤样本活检显示颗粒细胞层分裂。患儿予静脉注射萘夫西林治疗，随后的几天患儿状态逐渐改善。患儿入院7天后皮疹完全消退出院。,A six-year-old boy was hospitalized with a rash and fever. He had been well until two days before admission, when general pruritus developed. That evening, small water blisters developed at the base of his nose and face. During the next two days, the lesions spread despite treatment with cephalexin, which had been initiated one day before admission. The childs temperature was 38.3C on admission. He appeared extremely uncomfortable but not acutely ill. He had numerous flaccid bullae on his face, neck, axilla, perianal region, upper back, and thighs. Nikolskys sign was present (ready separation of the outer layer of epidermis from the basal layer), and a biopsy of a skin specimen revealed that the level of cleavage was at the granular layer. The boy was treated with intravenous nafcillin, and his condition improved over the next few days. He was discharged after seven days, with almost complete resolution of his rash.,临床表现,入院第1天前臂可见表皮脓疱和水疱,入院第4天，随着SSSS进展，全身及躯干 可见皮肤广泛的起疱和糜烂,入院第6日足部表皮起疱和糜烂,临床表现,Macular rash with Nikolskys sign,Evolution to scaly phase, more pronounced at periorificial areas.,临床表现,Staphylococcal scalded skin syndrome. Characteristic desquamation in gravely ill woman.,Staphylococcal scalded skin syndrome: diagnosis and management in children and adults,M.Z. Handler, R.A. Schwartz*,临床表现,Staphylococcal scalded skin syndrome after intra-articular injection of hyaluronic acid,Mod Rheumatol (2011) 21:316319,临床表现,Case 1 on day of admission, showing round ulceration on forehead, oval skin lesion on left cheek (A), and erosion on abdomen (B).,案例1入院当日显示，前额溃疡形成，左侧面颊椭圆形皮肤缺损，下腹部糜烂。,临床表现,Case 1 three days after admission, showing a new round pink injury on nose (A), crusty lesions on right ear (B),病例1入院后3天，显示鼻子处一个新的粉红色皮损，右耳硬性皮损。,临床表现,Case 1 three days after admission, showing left aspect of neck (C) and abdomen (D).,病例1入院后第3天显示左侧面颊和腹部硬性皮损。,临床表现,Staphylococcal Scalded Skin Syndrome in a newbornwith generalized bullous epidermolysis.,临床表现,临床表现,A newborn, age 17 days and 20 hours, (A) was admitted a few hours after the diagnosis of SSSS. Skin erythema and exfoliating rash were seen on the face and subsequently diffused to the whole body. Blistering and positive Nikolskys sign and the discomfort of the newborn were obvious. (B) Three days later the erythema subsided and the blisters dried up and crusted. (C) The same patient 11 days later, with intact skin and no scaring. The childs parents consented to the publication of this picture.,临床表现,Mild staphylococcal scalded skin syndrome: an underdiagnosed clinical disorder,临床表现,治疗前,治疗后,发病机制,病理机制是金黄色葡萄球菌产生的表皮剥脱毒素（exfoliative toxin，ET），使表皮细胞间桥粒溶解而出现尼氏征阳性。 金黄色葡萄球菌产生的ET是否能引起表皮细胞间桥粒分解，黏附作用消失与两者相互接触时间的长短及剥脱毒素量的多少有关。 此毒素可通过蛋白水解过程发挥作用，作用于表皮颗粒层，造成皮肤损伤。,Aalfs A S，Oktarina D A，Gilles G F，et al. Staphylococcal scalded skin syndrome:loss of desmoglein 1 in patientskinJ. Eur J Dermatol，2010，20（4）:451-456.,发病机制,该病的临床表现型与毒素血清分型相关。 表皮剥脱毒素作为一种丝氨酸蛋白酶，能作用于表皮颗粒层，特异性结合并水解桥粒芯糖蛋白1， 从桥粒芯糖蛋白1的胞外区性结合并水解桥粒芯糖蛋白1， 从桥粒芯糖蛋白1的胞外区疱。 表皮剥脱毒素还可能作为超抗原参与发病。 目前已发现并证实有4中ET存在：ETA、ETB、ETC、和ETD。其中ETA、ETB与人类的SSSS关系密切。,病理,Photomicrograph showing intraepidermal splitting at the stratum granulosum (haematoxylin and eosin; original magnificationx100.,显微镜检显示：表皮内颗粒细胞层裂开（苏木精- 伊红染色：原始片放大100倍）,病理,Biopsy of skin from the upper arm showing a subcorneal. intra-epidermal split consistent with staphylococcal scalded skin syndrome (day 4, H&E).,Australasian Journal of Dermatology (2002) 43, 199201,Staphylococcal scalded skin syndrome complicating acute generalized pustular psoriasis,病理,Histology of skin biopsy specimen (H&E staining) shows an epidermal detachment in the uppermost layer. Histological findings are consistent with staphylococcal scalded skin syndrome (SSSS),Mod Rheumatol (2011) 21:316319,Staphylococcal scalded skin syndrome after intra-articular injection of hyaluronic acid,病理,Skin biopsy showing loss of the stratum corneum and granular layer, with some acanlholysis of residual cells (x 150).,Staphylococcal scalded skin syndrome. Skin histopathology revealing intraepidermal cleavage through the stratum granulosum with little dermal inflammation. (Haematoxylin and eosin, original magnification 9400).,诊断,SSSS的诊断主要依据临床皮损表现，患者皮肤首先出现水肿性红斑，多始发于眼周、口周或面颈部，迅速蔓延至躯干及四肢近端，甚至泛发全身，并在红斑基础上发生松弛性水疱、大疱，以皮肤皱褶处（颈部、腹股沟、腋下及肛周）较严重，皮损处有明显触痛。 疱液及血液金葡菌培养阳性。,临床分型,可分为3种类型： （）泛发型：面部、颈部出现红斑，几小时到数日迅速蔓延至全身，水肿性红斑上有松弛性大疱，有明显触痛，Nikolsky征阳性，皮损逐日加重，2-5d后口周、眼周和背部表皮片状剥脱，形似烫伤创面，出现潮湿发亮的裸露面。 （）局限型：表现为松弛性大疱，可伴有局限性触痛性红斑，Nikolsky征阳性。 （）顿挫型：可表现为全身性或局限性触痛性红斑，可见帕氏线，无杨梅舌、黏膜疹及口周环状苍白圈，Nikolsky征阴性。,鉴别诊断,临床上SSSS需与非金葡菌中毒性表