儿童 Stevens-Johnson 综合征致失明1例

感染是儿童Stevens-Johnson综合征（ SJS）的重要诱因，眼部损害是主要的后遗症。本文报道1例可能与肺炎支原体和单纯疱疹感染相关的SJS，愈后遗留慢性眼部损害导致失明。患儿，男，9岁，全身弥漫分布红斑、水疱、糜烂，口、眼、肛周、尿道口周围粘膜糜烂、渗出，伴持续高热1个月。肺炎支原体IgM阳性，单纯疱疹病毒（HSV）1和2型IgG均阳性，给予阿奇霉素、阿昔洛韦、激素、静脉用丙种球蛋白（ IVIG）等治疗后皮疹消退，但口、眼炎症渗出仍明显，伴持续高热，停用上述药物，再次给予 IVIG冲击，起病第28天体温降至正常，口、眼粘膜愈合，但左眼失明，右眼视力下降。     

儿童Stevens-Johnson综合征致失明1例

感染是儿童Stevens-Johnson综合征(SJS)的重要诱因,眼部损害是主要的后遗症。本文报道1例可能与肺炎支原体和单纯疱疹感染相关的SJS,愈后遗留慢性眼部损害导致失明。患儿,男,9岁,全身弥漫分布红斑、水疱、糜烂,口、眼、肛周、尿道口周围粘膜糜烂、渗出,伴持续高热1个月。肺炎支原体Ig M阳性,单纯疱疹病毒(HSV)1和2型Ig G均阳性,给予阿奇霉素、阿昔洛韦、激素、静脉用丙种球蛋白(IVIG)等治疗后皮疹消退,但口、眼炎症渗出仍明显,伴持续高热,停用上述药物,再次给予IVIG冲击,起病第28天体温降至正常,口、眼粘膜愈合,但左眼失明,右眼视力下降。     

慢性湿疹和荨麻疹患者血清过敏原检测

报告了2000年3月－2001年3月对128例慢性湿疹，慢性荨麻疹患者进行血清过敏原特异性IgE抗体检测，并与40名正常人对照。结果128例患者血清过敏原反应阳性97例，对一种过敏原呈阳性反应24例，对2种以上过敏原呈阳性反应73例。阳性率较高的过敏原是屋尘，尘螨，多价霉 ，牛羊肉。40名正常人血清过敏原反应全部阴性。血清总IgE值：128例患者有94例＞50IU／mL，对照组均＜20IU／mL。     

121例泌尿生殖道感染患者支原体药敏结果分析

目的：了解STD门诊患支原体感染的耐药情况。方法：对121例STD门诊患支原体培养阳性标本进行药敏试验，检测支原体对10种抗生素的药物敏感性。结果：121例支原体阳性患中解脲支原体(Uu)、人型支原体(Mh)及解脲、人型支原体混合感染阳性率分别为83．4％、4．9％、11．5％。Uu对10种药物敏感性最高是交沙霉素98％，其次是阿奇霉素93．9％。耐药性最高为四环素31．9％，其次是强力霉素和美满霉素19．5％。结论：STD门诊支原体感染主要为Uu引起(占83．4％，101／121)；交沙霉素是治疗泌尿生殖道Uu感染的首选药物。     

泌尿生殖道感染支原体培养及耐药情况分析

目的了解解脲支原体（UU）和人型支原体（MH）的感染及耐药情况，指导临床合理用药。方法采用试剂盒对98l例泌尿生殖道标本进行体外培养。结果981例支原体培养中阳性411例（41．9％），其中UU阳性384例（39．1％），MH阳性2例（0．2％），UU＋MH阳性25例（2．5％）。男性支原体阳性124例（30．2％），女性阳性287例（69．8％），女性支原体培养阳性率显著高于男性（x^2=18．048，P〈0．001）。对支原体敏感的药物分别为美满霉素（89．0％）、强力霉素（88．8％）、克拉霉素（56．2％）、司帕沙星（45．3％），对环丙沙星、氧氟沙星、交沙霉素、罗红霉素、壮观霉素、阿奇霉素的耐药率分别为71．8％、50．6％、40．6％、37．0％、30．7％、30．4％。结论对泌尿生殖道支原体感染可首选美满霉素和强力霉素，不宜选用环丙沙星、氧氟沙星和交沙霉素。     

HIV阴性神经梅毒18例临床分析

目的探讨神经梅毒的临床特点、实验室检查、治疗与预后的关系。方法18例神经梅毒的临床资料进行回顾性分析。结果18例神经梅毒患者中男16例,女2例;平均发病年龄50．6岁,其中麻痹性痴呆9例,其次为脊髓痨3例,无症状型2例,视神经炎2例,脑膜型1例,脑膜血管型1例。血清快速血浆反应素环状卡片试验、梅毒螺旋体颗粒凝集试验均为阳性,脑脊液检查：lO例快速血浆反应素环状卡片试验阳性,18例梅毒螺旋体颗粒凝集试验均为阳性,10例蛋白定量〉500mg／L;12例白细胞数≥10×10^9／L,CT、磁共振成像、肌电图等均无特异性,首诊误诊率为77．8％。结论神经梅毒临床表现复杂多样,误诊率高,预后较差,应重视脑脊液检查。     

甲氨蝶呤对寻常型银屑病患者外周血MMP-2表达的调控作用

目的：探讨甲氨蝶呤（methotrexate,MTX）对寻常型银屑病患者外周血中基质金属蛋白酶2（matrix metalloproteinase 2,MMP-2）表达的调控作用。方法：采用酶联免疫吸附试验（ELISA）技术检测19例寻常型银屑病外周血治疗前,MTX治疗8周后及19例正常对照者外周血MMP-2的表达。结果：MMP-2在寻常型银屑病外周血中治疗前的表达水平为（268．94±43．15）ng／mL,经过MTX治疗8周后的表达水平为（228．67±50．32）ng／mL,正常对照者的表达水平为（213．27±53．04）ng／mL。MMP-2在寻常型银屑病外周血治疗前的表达水平明显高于治疗后及正常对照者的表达水平（P〈0．01）,治疗后的表达水平与正常对照者的表达水平差异无统计学意义（P〉0．05）。结论：MTX能抑制银屑病患者外周血MMP-2的表达,这可能是MTX治疗银屑病的作用机制之一。     

注射精制抗狂犬病血清致过敏反应及血清病各3例

6例中男女各3例。注射的精制抗狂犬病血清制剂系卫生部兰州生物制品研究所生产，规格为700IU／支，批号为940701号。按常规作过敏试验，阴性者分3次脱敏注射仍无反应，按40IU／kg注射，特别严重者注射80～100IU。3例女患者在注射抗狂犬病血清10min左右出现烦躁，脸色苍白、胸闷、出汗、轻度恶心腹痛、脉搏细弱、血压正常。其中2例（成人儿童各1例）平卧体息后自行缓解。1例儿童较重即注射0．25mg肾上腺素后逐渐缓解。另3例注射精制抗狂犬病血清7天后发热（38℃），注射局部出现浮肿，关节疼痛，皮疹为黄豆大小暗红色风团，瘙痒，指压不退…     

丘疹紫癜性手套和短袜样综合征1例

患者女,17岁,发热3 d,手足部起红色皮疹2 d。皮肤科情况:手足背部可见红斑、小丘疹和瘀点,皮疹分布呈手套和短袜状。实验室检查:微小病毒B19 IgM抗体阳性,但IgG抗体阴性。诊断:丘疹紫癜性手套和短袜样综合征。对症治疗4 d后皮损消退。     

抗Jo-1抗体与皮肌炎和多发性肌炎临床关系的探讨

目的探讨抗Jo-1抗体与皮肌炎和多发性肌炎（PM／DM）的临床关系。方法分析16例抗Jo-1抗体阳性的多发性肌炎／皮肌炎（PM／DM）的临床特征,并与81例阴性组进行比较。结果本组中抗Jo-1抗体阳性占16．5％,抗Jo-1抗体阳性组出现肺间质病变（ILD）11例（68．75％）、肺部感染15例（93．75％）、和多关节炎／关节痛8例（50．00％）、肌肉活检异常3例（18．75％）、肌力异常4例（25．00％）、RF阳性5例（55．50％）、技工手4例（25．00％）、发热14例（87．50％）、皮肤硬肿5例（31．25％）与抗Jo-1抗体阴性组比较,差异显著（P〈0．05）。结论在多发性肌炎／皮肌炎（PM／DM）中,抗Jo—1抗体阳性者有明显的肺间质病变、肺部感染、多关节炎、RF阳性、技工手、发热、皮肤硬肿等临床特征,提高对Jo-1抗体综合征的认识,早期诊断,早期治疗,提高生存率。     

Stevens-Johnson syndrome: a diagnostic challenge in the absence of skin lesions

We report a 14-year-old boy with Stevens-Johnson syndrome who presented with mucous membrane lesions but without skin lesions and discuss the differential diagnosis of oral mucous membrane lesions in childhood. Stevens-Johnson syndrome in children is most frequently caused by a Mycoplasma pneumoniae infection. The full clinical picture of Stevens-Johnson syndrome can be present before seroconversion of Mycoplasma antibodies is observed. One should keep in mind that one negative titer of Mycoplasma antibodies does not rule out M. pneumoniae infection.     

Cold urticaria and virus infections: a clinical and serological study in 39 patients

Thirty-nine patients with cold urticaria seen over a 12-year-period were re-examined. All but 12 still had positive skin tests for cold and only five of these had shown a spontaneous cure. Fourteen patients were prone to collapse on cold exposure. The incidence of atopy in this group was comparable to that in control groups. Cold urticaria is an extremely chronic disease. The mean disease duration was 9.3 years. Serum antibodies to Epstein-Barr virus, measles virus, cytomegalovirus (CMV), varicella-zoster virus (VZV), herpes simplex virus (HSV), Chlamydia psittaci and Mycoplasma pneumoniae were determined in all 39 patients and compared with control groups. The EBV-antibody patterns (heterophile antibodies and different types of EBV-specific antibodies) showed no evidence of current or of recent primary or secondary infection with EBV. Complement fixing antibody titres to measles virus, CMV, HSV and Mycoplasma pneumoniae were significantly higher in cold urticaria patients than in controls. The existence of a basic immuno-regulatory defect responsible for both the cold urticaria and the elevated antibody levels is proposed.     

Effect of high-dose intravenous immunoglobulin therapy in Stevens-Johnson syndrome: a retrospective,multicenter study

BACKGROUND:Stevens-Johnson syndrome (SJS) is a severe cutaneous drug reaction associated with considerable morbidity, possible transition to toxic epidermal necrolysis (TEN) and death in certain cases. OBJECTIVE: To determine whether treatment with high-dose IVIG in SJS patients may improve outcome. METHODS: Data from 12 patients (collected between January 1997 and November 2000 from 7 university dermatology centers in Europe and North America) diagnosed with SJS according to a recent consensus definition was analyzed retrospectively. All patients had progressive ongoing epidermal detachment at the time of treatment initiation. Patients with overlap syndromes and TEN were excluded. Tolerance, survival at 45 days after onset and total healing time were assessed. RESULTS: Twelve SJS patients (mean age 44 years) were treated with IVIG at a mean dose of 0.6g/kg/day for an average of 4 days. An objective response to IVIG infusion was observed in all patients within a mean of 2 days, and the overall survival rate was 100%. Total skin healing occurred, on average, within 8.3 days. Time to total healing was shorter in a group of patients with fewer severe underlying diseases who had received IVIG infusion rapidly after the onset of skin lesions. CONCLUSION: High-dose IVIG may be effective in blocking the progression of SJS and reducing the time to complete skin healing.     

肺炎支原体诱导的皮疹和黏膜炎8例临床特征及预后分析

【摘要】 目的 分析肺炎支原体诱导的皮疹和黏膜炎（MIRM）的临床特征及预后。方法 调阅中山大学附属第一医院2004年11月至2021年5月出院诊断为多形红斑/重症多形红斑或Stevens-Johnson综合征患者的资料,以MIRM诊断标准筛选出其中的MIRM患者,且排除了其他病因,分析其临床表现、实验室和辅助检查、治疗和预后。结果 8例符合MIRM诊断,其中男4例,女4例,发病年龄4 ~ 30（15.63 ± 9.16）岁。8例均有发热,其中5例有咳嗽、咽痛等上呼吸道前驱症状。所有患者均有口腔黏膜损害,其中5例有口唇血痂;7例有眼损害,表现为结膜充血及分泌物增多。所有患者均有皮损,表现为靶形损害5例、水疱4例。所有患者血清学肺炎支原体IgM均阳性。1例反复出现干咳等上呼吸道感染,每次发作与肺炎支原体感染密切相关,取外周血行全外显子测序显示,NLRC4和IRGM杂合突变。3例患者行皮损组织病理检查,符合多形红斑。7例系统使用糖皮质激素治疗,6例静脉注射免疫球蛋白,5例阿奇霉素,5例使用阿昔洛韦或伐昔洛韦或利巴韦林。平均随访2.9年,3例痊愈,1例失明,1例反复出现干咳和口腔溃疡及四肢皮疹,余3例分别出现眼睑板腺功能障碍、泪点狭窄及角膜上皮损害等眼部损害。结论 MIRM好发于儿童及年轻成人,多有发热、咽痛、咳嗽等前驱症状,黏膜损害明显,部分有皮肤靶形损害。多数患者单次发病后痊愈,个别反复发作者可能与自身炎症相关基因和感染相关基因突变有关。     

Mycoplasma pneumoniae–Associated Mucocutaneous Disease in Children: Dilemmas in Classification

There is controversy regarding precise definitions for Stevens–Johnson syndrome (SJS) and erythema multiforme (EM) major because of overlap in clinical presentations. SJS and EM major associated with Mycoplasma pneumoniae have been reported to occur in children, but Mycoplasma is more commonly reported with SJS. We sought to further characterize Mycoplasma‐associated mucocutaneous disease. Through retrospective chart review over 10 years, six children hospitalized with a diagnosis of SJS who also tested positive for Mycoplasma infection were reviewed. Using documented physical examinations and photographs, diagnoses of SJS or EM major were retrospectively made based upon cutaneous lesional morphology employing the classification system proposed by Bastuji‐Garin et al. The majority of patients were boys, with limited acral cutaneous lesions. All patients required prolonged hospitalization because of mucosal involvement and had good short‐term outcomes. When the classification system was retrospectively applied, five of the six patients were reclassified with a diagnosis of EM major instead of SJS. Children with Mycoplasma‐associated EM major and SJS in our small retrospective series appeared to have significant mucosal involvement but more limited cutaneous involvement with lesional morphology, which is more characteristic of EM major.     

Pityriasis rosea Gibert and Mycoplasma pneumoniae infection

In the patients with pityriasis rosea (Gibert), who came to our hospital in the period from November of 1979 to May of 1981, cold hemagglutinin (CHA), complement fixation (CF) and indirect hemagglutination (IHA) antibodies against Mycoplasma pneumoniae were observed. In 6 out of 30 cases, the CF titer was 1: 32 or higher and CHA 1: 128 or higher, indicating the presence of Mycoplasma pneumoniae infection; 22 cases were serologically positive and only 8 cases were negative for Mycoplasma pneumoniae. These results suggest that cases with pityriasis rosea may possibly be caused by Mycoplasma pneumoniae infection, although not all cases are serologically positive.     

Histopathological and epidemiological characteristics of patients with erythema exudativum multiforme major, Stevens—Johnson syndrome and toxic epidermal necrolysis

Summary The clinical and histopathological classification of erythema exudativum multiforme major (EEMM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are difficult, due to the lack of clear-cut criteria. Based on a new clinical classification, 149 of 219 (68%) histopathological specimens, from a total of 534 patients with EEMM. SJS and TEN, have been reviewed. A comparison was made with the clinical picture, and any past history of infection or drug intake. All patients had been included in the German Registry of Severe Skin Reactions between April 1990 and December 1993. No differences could be found between the biopsies examined and the total number of histopathological specimens, concerning clinical diagnosis, gender and age. Sections from 28 of 149 specimens were not diagnostic or were too old to be properly evaluated. In nine cases, other diagnoses were proposed. One hundred and eleven of the histological slides with the diagnosis of EEMM (n= 16), SJS (n=34) and TEN (n=61), were classified as epidermal type of erythema multiforme. In these 111 slides, necrotic keratinocytes could be found, ranging from individual cells to confluent epidermal necrosis. The epidermo-dermal junction showed changes ranging from vacuolar alteration up to subepidermal blisters. The dermal infiltrate was superficial and mostly perivascular. It was sparse in SJS and TEN, and more pronounced in EEMM. Oedema in the papillary dermis was evident occasionally in all clinical groups. In 59 of 111 cases (53%), at least one eosinophil was present in the dermis. In 11 of 111 (10%), more than 10 eosinophils per field could be seen. Eosinophils were less common in the patients with the most severe forms of TEN, in whom there was detachment of more than 30% of the skin surface area. No differences in the history for drug intake, or for infection with Mycoplasma pneumoniae, herpes simplex and other organisms, could be detected between patients with or without eosinophils in their skin sections. This dermatopathological study of patients with EEMM. SJS and TEN indicates that the epidermal type of erythema multiforme is the pathological correlate for these diseases.     

Erythema multiforme majus and Chlamydia pneumoniae infection

BACKGROUND: Erythema multiforme majus of infectious origin is an acute eruptive syndrome seen more commonly in young subjects and characterised by an appearance of round target lesions. In most cases, it is associated with infection involving Herpes simplex virus or Mycoplasma pneumoniae. We report an original case of erythema multiforme majus subsequent to infection with Chlamydia pneumoniae. CASE REPORT: An 18 year-old man was hospitalised for management of generalised skin rash comprising lesions in rings, associated with bullous and post-bullous lesions, chiefly in the oral (preventing eating) and genital areas in a setting of febrile cough. Various bacterial agents (Mycoplasma pneumoniae, Chlamydia pneumoniae) and viral agents were suspected, but serological testing for Chlamydia pneumoniae alone was positive with IgM of 128 IU and IgG of 64 IU. The outcome was favourable within several days following administration of symptomatic treatment (rehydration, mouthwashes, etc.) and aetiological treatment (acyclovir: 30 mg/kg/d, ofloxacine: 400 mg/d). At D15, serologic tests for Mycoplasma pneumoniae continued to be negative. Anti-Chlamydia pneumoniae IgM and IgG were 256 IU. At D30, IgM was 128 IU while IgG remained at 256 IU. DISCUSSION: The existence of a systematic skin rash comprising typical target lesions and mucosal lesions in the oral and genital areas suggested to us a diagnosis of erythema multiforme majus. Screening for the agents generally responsible was negative and drug-induced rash was ruled out. Serological tests for Chlamydia pneumoniae were positive at various times, resulting in diagnosis of erythema multiforme majus secondary to infection with Chlamydia pneumoniae. Following demonstration of the presence of Chlamydia pneumoniae using reliable methods and the elimination of other causes of erythema multiforme majus, dermatologists should opt for this aetiology in order to optimise treatment.     

Mycoplasma pneumoniae-Associated Mucositis with Minimal Skin Manifestations

Mycoplasma pneumoniae-associated mucositis is a rarely described complication of M. pneumoniae infection presenting with ocular, oral, and genital involvement but without the typical skin lesions seen in Stevens-Johnson syndrome. A 27-year-old man with a past history of asthma presented at the emergency room with a 1-week history of cough (initially non-productive but subsequently associated with non-bloody mucopurulent sputum), fever, myalgias, headache, and progressive dyspnea. Two days before admission he had commenced amoxicillin/clavulanic acid with no improvement. The patient reported bilateral conjunctival injection and hemorrhagic ulcers on the lips commencing the day prior to admission. Physical examination revealed fever (39 degrees C), bilateral exudative conjunctivitis, painful hemorrhagic ulcers on the lips, tongue, and oral mucosa, small scrotal erosions, erythema of the penile meatus, and small erythematous bullae on the dorsum of each hand; subsequently, the patient developed bullae at the venipuncture site on his right arm. Laboratory tests revealed positive IgM serology for M. pneumoniae, with titer elevation. The patient was successfully treated with levofloxacin and prednisolone. Our case appears to be the first adult patient described with M. pneumoniae-associated mucositis, which has previously been reported only in pediatric patients. This is also the first reported instance of a case of M. pneumoniae-associated mucositis treated with levofloxacin and prednisolone. M. pneumoniae infection should be considered in all cases of mucositis, and treatment of this condition with levofloxacin and prednisolone seems to be effective.     

A multicentre study to determine the value and safety of drug patch tests for the three main classes of severe cutaneous adverse drug reactions

Background Drug patch tests (PTs) can reproduce delayed hypersensitivity to drugs and entail a moderate re‐exposure of patients to offending drugs. Objectives To determine the value of PTs for identifying the responsible drug in severe cutaneous adverse drug reactions (SCARs) such as acute generalized exanthematous pustulosis (AGEP), drug reaction with eosinophilia and systemic symptoms (DRESS) and Stevens–Johnson syndrome/toxic epidermal necrolysis (SJS/TEN). Methods In a multicentre study, PTs were conducted on patients referred for DRESS, AGEP or SJS/TEN within 1 year of their SCAR. All drugs administered in the 2 months prior to and the week following the onset of the SCAR were tested. Results Among the 134 patients included (48 male, 86 female; mean age 51·7 years), positive drug PTs were obtained for 24 different drugs. These included positive tests for 64% (46/72) of patients with DRESS, 58% (26/45) of those with AGEP and 24% (4/17) of those with SJS/TEN, with only one relapse of AGEP. The value of PTs depended on the type of drug and the type of SCAR (e.g. carbamazepine was positive in 11/13 DRESS cases but none of the five SJS/TEN cases). PTs were frequently positive for beta lactams (22 cases), pristinamycin (11 cases) and in DRESS with pump proton inhibitors (five cases), but were usually negative for allopurinol and salazopyrin. Of 18 patients with DRESS, eight had virus reactivation and positive PTs. In DRESS, multiple drug reactivity was frequent (18% of cases), with patients remaining sensitized many years later. Conclusions PTs are useful and safe for identifying agents inducing SCAR.