儿童金黄色葡萄球菌烫伤样皮肤综合征课件.pptx

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1、儿童金黄色葡萄球菌烫伤样皮肤综合征Staphylococcal Scalded Skin SyndrometheRittertheRitter sdiseasesdisease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 ma

2、ny 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。葡葡萄萄球球菌菌中中毒毒性性

3、休休克克综综合合征征（STSSSTSS）和金金黄黄色色葡葡萄萄球球菌菌烫烫伤伤样样皮皮肤肤综综合合征征（SSSSSSSS）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

4、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

5、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

6、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 fev

7、er.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 gen

8、eral 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 unc

9、omfortable 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 cleavagewas at the

10、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 signEvoluti

11、on to scaly phase,more pronounced at periorificial areas.临床表现Staphylococcal scalded skin syndrome.Characteristicdesquamation in gravely ill woman.Staphylococcal scalded skin syndrome:diagnosis andmanagement in children and adultsM.Z.Handler,R.A.Schwartz*临床表现Staphylococcal scalded skin syndrome after

12、 intra-articularinjection of hyaluronic acidMod 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 in

13、jury 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 newbornwithgeneralized bullous epidermolysis.临床表现临床表现A newborn,age 17 d

14、ays 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 subside

15、d 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 thepublication 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 to

16、xin，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

17、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

18、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)43,199201Staphylococcal scalded skin syndrome complicating acute generalized pustular psoriasis病理Histology o

19、f 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)21:316319Staphylococcal scalded skin syndrome after intra-articular injection of hyaluronic acid病理Skin biops

20、y 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 magnific

21、ation 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。临床分型可分为可分为3 3种类型：种类型：（）泛泛发发型型：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。（）局局限限型型：表现为松弛性大疱，可伴有局限性触痛

22、性红斑，Nikolsky征阳性。（）顿顿挫挫型型：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皮坏死松解症（toxic epidermal necrolysis，TEN）相鉴别。TEN主要发生于岁以上患儿，发病原因主要为药物性，属变态反应性疾病，皮肤触痛轻，Nikolsky征仅皮损处阳性，黏膜损害严重，治疗困难，病程长达1-3周，病死率高。治疗金黄色葡萄球菌性烫伤样皮肤综合征诊断明确患者，须尽可能早使用足量确切敏感抗生素，治疗上是非常重要的，首选-内酰胺类抗生素。目前耐甲氧西林葡萄球菌（MRS

23、A）的感染率不断增加，已发现MRSA引起的SSSS，部分选用强有力敏感万古霉素抗感染治疗，可明显缩短疗程，病情恢复快，未见明显副作用的产生。有学者认为目前万古霉素应列为治疗SSSS的首选药物。尤其是重症或是其他药物疗效不佳时，建议要及时应用万古霉素。治疗在SSSS治疗中，国内外对糖皮质激素的使用也一直存在争议。糖皮质激素具有抗炎、调节免疫的作用，但糖皮质激素本身有引起免疫抑制加重感染、干扰肾上腺皮质功能、诱发溃疡等诸多副作用。研究结果表明首先控制感染病灶，消除或减少细菌产生的外毒素，同时应用糖皮质激素以抗炎，缩短疗程。可能与糖皮质激素较强的抗炎作用、有效减少可溶性剥脱毒素（ET）的致病作用有关。但应严格掌握用药剂量，病情控制后，应及时停药。治疗部分病情较重患者可给予静脉滴注丙种球蛋白针300-500mg/（kgd），连用3-5d，均取得满意疗效。合理高效的护理是康复的关键。潮红部位外用炉甘石洗剂。注意保暖，穿绵软宽松衣服。接触患儿要动作轻柔。注意翻身，避免背部压迫糜烂，继发感染。口周皮损影响患儿进食时，可以喝温热的鲜牛奶等流食。Thank you for your attention.感谢您的关注！