临床结合疱顶表皮病理鉴别诊断葡萄球菌性烫伤样皮肤综合征

目的 探讨鉴别葡萄球菌性烫伤样皮肤综合征及中毒性表皮坏死松解症的可靠方法.方法 观察并比较12例葡萄球菌性烫伤样皮肤综合征患者及6例中毒性表皮坏死松解症患者的临床表现和治疗情况,并利用患者皮损的疱顶表皮切片染色,了解其组织学表现.结果 葡萄球菌性烫伤样皮肤综合征的疱顶表皮组织病理均表现为由角质层和小部分残余颗粒层细胞组成.均未见表皮其他层次细胞.中毒性表皮坏死松解症患者的疱顶表皮组织病理均表现为疱顶表皮由表皮全层组成.角质形成细胞呈明显嗜酸性变性坏死;两组患者的疱顶表皮组织病理表现差异显著,临床诊断与疱顶表皮组织病理表现完全一致.结论 疱顶表皮组织病理检查可作为鉴别葡萄球菌性烫伤样皮肤综合征和中毒性表皮坏死松斛症的方法之一.     

葡萄球菌烫伤样皮肤综合征16例临床分析

葡萄球菌烫伤样皮肤综合征（ＳＳＳＳ）是一种少见而严重的金黄色葡萄球菌引起的感染性疾病，主要发生于５岁以下的婴幼。报首幼儿及儿童病例共１６例，均有典型的临床皮肤症状，经早期应用抗生素治疗，１６例患儿均获痊俞。本文就ＳＳＳＳ的病因、临床表现、病理、鉴别诊断及治疗作了讨论。     

中毒性表皮坏死松解症合并噬血细胞综合征一例

患儿女,4岁,因全身泛发红斑、水疱伴发热3 d,于2018年4月3日至福建医科大学附属第一医院皮肤科就诊。患儿入院前3天因上呼吸道感染服用氨酚伪麻那敏口服液,退热后,躯干、颈部、耳后出现少许瘙痒性红斑,伴反复高热,最高体温42 ℃,皮损逐渐蔓延全身,面颈部、躯干红斑基础上出现大小不等的水疱,部分水疱及口腔黏膜破溃成糜烂面。无乙型肝炎、结核病史,既往无药物及食物过敏史……     

A case of toxic epidermal necrolysis with lesions mostly on sun-exposed skin

Toxic epidermal necrolysis (TEN) is a cutaneous, mostly drug-induced reaction pattern characterized by a widespread necrosis and exfoliation of epidermis, involving more than 30% of the body surface area. Photo-induced Stevens-Johnson syndrome and erythema multiforme, which belong to the same spectrum of TEN, have been described before. However, there is only one case of TEN limited to sun-exposed areas, which was caused by clobazam. In this report a second case of TEN because of naproxen, with lesions showing clear-cut borders on sun-exposed body areas resembling a sun-burn reaction, is presented.     

小儿葡萄球菌烫伤样皮肤综合征18例临床分析

目的：了解小儿葡萄示菌烫伤样皮肤综合征（SSSS）的临床特点及治疗措施。方法：回顾性分析1994年10月-2001年3月间18例9d-3岁小儿SSSS的临床表现，治疗措施及其疗效。结果：全部均有泛发性红斑及皮肤触痛；除3例无表皮剥脱及尼氏征阴性之外，余者的剥脱面积为5%-69.5%(平均26.8%)。7例作创面分泌物细菌培养，仅1例金黄色葡萄球菌阳性，及时联用新青霉素Ⅱ与头孢菌素，适当的局部处理使裸露创面在7-10d完全愈合，全部痊愈出院。结论：SSSS好发于新生儿及婴幼儿，母乳喂养似对发病无保护作用。早期诊断，及时应用耐β-内酰胺酶抗生素及适当的局部处理是治疗的关键。     

Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of treatment options

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous reactions that are medication-induced in most instances. While the clinical manifestations of SJS and TEN are well-defined, the optimal treatment for these disorders is not. Case reports have shown benefit with the use of a variety of agents including tumor necrosis factor-alpha inhibitors and cyclophosphamide, whereas thalidomide was associated with an increased mortality. Plasmapheresis and cyclosporine have also demonstrated efficacy anecdotally, albeit with an even smaller number of cases in the literature. Most of the reporting has focused on the use of systemic corticosteroids and intravenous immunoglobulin (IVIG) for these severe reactions. The majority of studies analyzing the use of IVIG in the treatment of SJS/TEN show a benefit, though more recent series cast doubt upon this conclusion. The results of these studies are summarized in this present review study.     

Update on pathobiology in Stevens-Johnson syndrome and toxic epidermal necrolysis

Stevens-Johnson syndrome (SJS) and toxic epidermal necrosis (TEN) are rare but life-threatening severe cutaneous adverse reactions (SCARs), which are mainly induced by a variety of drugs. Once considered to be unpredictable, significant progress has been achieved in understanding the pathological mechanisms underlying such reactions. Recent studies suggested that SJS/TEN is a specific immune reaction where human leukocyte antigen (HLA) alleles specific for certain drugs in defined populations are involved in the activation of cytotoxic T lymphocytes (CTLs) and natural killer (NK) cells. Upon the activation, various cytotoxic and immunological signals, including but not limited to Fas/Fas ligand, perforin/granzyme B, and granulysin are launched to mediate the disseminated keratinocyte death in SJS/TEN. This review provides an update on the pathobiology of SJS/TEN in both the genomic and immunologic perspectives. The knowledge gained from these cutting-edge studies will form the basis for better prevention and management of SJS/TEN.     

Clinical risk management of Stevens–Johnson syndrome/toxic epidermal necrolysis spectrum

Clinical risk management concedes that risk is inherent to all health-care processes. Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but potentially life-threatening reactions to medications. Risk management should be considered prior to starting, during, and after therapy. Prior to starting therapy, risks that need to be assessed include any specific patient groups that may be at greater risk for the development of SJS/TEN. Gene testing is in place for Chinese and Thai patients who are going to be exposed to carbamazepine. During therapy, it is important to recognize SJS/TEN as a possible adverse drug reaction. Diagnostic criteria have changed, and more data exist on drugs with an increased risk. Although there is no standardized treatment for all patients with SJS/TEN, options that have been used include cyclosporine, corticosteroids, and intravenous immunoglobulin. Standards of care are usually defined locally, but new treatments, such as amniotic membrane support for ocular damage, may need to be considered. Good communication skills are needed to allow practitioners to show empathy and to provide disclosure. Risk management after a reaction includes skills in acknowledging bad outcomes or error; freedom to say "sorry" as defined by "apology laws," and knowing the rights provided by "Quality Assurance Conferences," where the information discussed is protected. In other words, the patient is best supported after an event like SJS/TEN if the practitioner is knowledgeable about optimal care standards and their legal rights and obligations.     

A case of eruptive lichen spinulosus after toxic epidermal necrolysis

We report a case of a 13-year-old boy who presented with eruptive monomorphic white papules on the trunk and arms involving regions previously affected by toxic epidermal necrolysis (TEN). Biopsy revealed compact keratin involving the hair follicle and sparse mixed perivascular infiltrate, findings consistent with lichen spinulosus. Improvement was noted after treatment with ammonium lactate 12% lotion. While cutaneous dyschromia and xerosis are common after TEN, lichen spinulosus has not yet been described in the literature. It is important for providers to be aware of any potential cutaneous sequelae of TEN that can affect quality of life in order to best counsel their patients.     

葡萄球菌性烫伤样皮肤综合征42例治疗分析

通过对我院2002年6月-2008年7月收住的42例葡萄球菌性烫伤样皮肤综合征(SSSS)患儿的回顾性分析,探讨有效治疗方案.根据皮损面积及全身情况将其分为轻、中、重3型,病情分级对临床治疗方案的实施及愈后有关键性的指导作用.轻型患儿单用抗生素即可;中度患儿在应用广谱抗生素的同时加用适量糖皮质激素,可加快病情恢复;重度患儿在应用广谱抗生素和适量糖皮质激素的同时加用静脉注射用人免疫球蛋白,可有效控制病情,缩短病程.     

葡萄球菌性烫伤样皮肤综合征的致病金黄色葡萄球菌剥脱毒素基因型及耐药研究

目的 探讨葡萄球菌性烫伤样皮肤综合征（SSSS）致病的金黄色葡萄球菌（简称金葡菌）分泌表皮剥脱毒素（ET）的主要类型及耐药情况。方法 108株金葡菌分离自SSSS（36例）、脓疱疮（36例）及皮肤脓肿（36例）。用多重PCR法测定致病金葡菌菌株产ETA、ETB、ETD基因型，Kirby-Bauer纸片法检测其对20种抗生素的敏感性。结果 36株来源于SSSS的金葡菌 100％ （36/36）为产ET菌株，其中单产ETA 2株（6％），ETB 7株（19％），兼产ETA和ETB 27株（75％）。来源于脓疱疮的36株金葡菌78％ （28/36）为产ET菌株，单产ETA 5株（14％），兼产ETA和ETB 23株（64％），未发现单产ETB金葡菌。而分离自皮肤脓肿的36株金葡菌仅2.8%（1/36）产ET，为兼产ETA和ETB菌株。108株金葡菌均未检测到ETD。金葡菌产ET的型别分布在三组疾病间差异有统计学意义（χ2 = 89.4，P ＜ 0.01），且分离自SSSS的金葡菌株产ET的比例明显高于脓疱疮组（χ2 = 9.0，P < 0.01）和脓肿组（χ2 = 68.1，P < 0.01）。三组病例的致病金葡菌对青霉素、氨苄西林、大环内酯类及克林霉素高度耐药，但对头孢类抗生素均敏感。脓肿组发现2例耐甲氧西林金葡菌株（MRSA）。结论 SSSS及脓疱疮患儿的致病金葡菌主要为产ET菌株，并以兼产ETA和ETB菌株为主。     

Open trial of ciclosporin treatment for Stevens–Johnson syndrome and toxic epidermal necrolysis

Background Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute mucocutaneous reactions associated with poor prognosis. The treatment is mainly symptomatic, based on supportive care. Until now, several curative treatments have been proposed without evidence of effectiveness. Objectives To evaluate the effect of ciclosporin on SJS and TEN after a short series had suggested a benefit. Methods We conducted an open, phase II trial to determine the safety and possible benefit of ciclosporin. Among the 45 consecutive patients admitted for SJS/TEN from March 2005 to September 2007, 29 fulfilled inclusion criteria. Ciclosporin was administered orally (3 mg kg^?1 daily for 10 days) and tapered over a month. Clinical and biological evaluations were performed sequentially. Predicted death rate was estimated with a validated prognostic score (SCORTEN). Results Twenty‐nine patients were included at a mean ± SD of 2·8 ± 1·8 days after onset. The final diagnosis was SJS (n = 10), SJS/TEN overlap (n = 12) and TEN (n = 7). One month of treatment was completed in 26. Ciclosporin was stopped after more than 10 days in three cases for side‐effects including posterior leucoencephalopathy (n = 1), neutropenia (n = 1) and nosocomial pneumopathy (n = 1). Ciclosporin dosage was tapered earlier than scheduled in two cases for alteration in renal function. The prognostic score predicted 2·75 deaths; none occurred (P = 0·1). Mean epidermal detachment remained stable in 18 of 29 cases (62%). The mean ± SD hospital stay was 16·2 ± 9·1 days. Conclusions Both the death rate and the progression of detachment seemed lower than expected, suggesting a possible usefulness of ciclosporin in SJS and TEN that needs to be confirmed.     

Low N-acetylating capacity in patients with Stevens-Johnson syndrome and toxic epidermal necrolysis*

Abstract Low constitutive N-acetylating capacity has been implicated as a predisposing factor for the development of adverse reactions to certain drugs. This prompted us to investigate whether the N-acetylating capacity of patients with serious cutaneous adverse reactions, i.e., Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) differed from that of healthy control subjects. N-acctylating activity was measured in hair root cells by preparing a homogenate from freshly extracted hair roots and assessing acetyl-CoA-dependent N-acetylation by RP-HPLC using 2-aminofluorene as a substrate. Samples were obtained from hospitalized patients suffering from acute SJS and TEN or from healthy controls. All patients with SJS and TEN were found to have a low N-acetylating capacity (0.85 nmol/mg/min compared to 2.21 nmol/mg/min in controls, p<0.05). Based on these findings, a low constitutive N-acetylating capacity may be one of the predisposing factors for the development of serious cutaneous adverse reactions to drugs that require N-acetylation in these patients.     

葡萄球菌性烫伤样皮肤综合征35例临床分析

目的：分析葡萄球菌性烫伤样皮肤综合征（staphylococcal scalded skin syndrome，SSSS）的发病年龄、诱发因素、临床表现、实验室检查、治疗及预后。方法：回顾性分析35例儿童金葡菌烫伤样皮肤综合征的临床资料。结果：35例病例中，有21例发生于3岁以内婴幼儿，约占总病例的60％；发病中位年龄为2岁5个月；皮损主要表现为触痛性红斑、松弛性大疱、表皮剥脱（100％），口周放射状皲裂（94．3％），发热（42．9％），结膜炎（37．1％），实验室检查血白细胞及c反应蛋白未见显著性升高。结论：SSSS近年来时有发生，婴幼儿触痛性红斑、松弛性大疱、表皮剥脱伴口周放射状裂纹时应高度怀疑本病。早期、足量、有效的使用抗生素，是治疗的关键，联合大剂量静脉注射免疫球蛋白，可明显缩短病程。