肺炎支原体感染引起的皮肤和黏膜损害2例分析

报告2例肺炎支原体感染引起的皮肤和黏膜损害。例1.患儿男,7岁。发热和咳嗽1周,嘴唇溃烂及结膜充血3 d。例2.患儿男,9岁。发热及咳嗽6 d,眼充血及口唇糜烂3 d。2例患儿均无明确药物过敏史,均有发热及呼吸道症状。皮肤科检查示双侧化脓性结膜炎、口腔黏膜及唇出血性糜烂,可见分散的非典型靶形皮损,其中例1患儿生殖器黏膜受累。2例患儿血清病原学检查均示肺炎支原体抗体阳性,胸部X射线检查提示非典型肺炎。2例患儿最终诊断为肺炎支原体感染引起的皮肤和黏膜损害。2例患儿均予静脉滴注阿奇霉素联合甲泼尼龙和大剂量静脉注射用人免疫球蛋白（IVIG）治疗,同时予支持性护理（黏膜和皮肤护理、补充液体以及疼痛管理）。治疗后2例患儿肺炎好转,皮肤和黏膜皮损完全消退,随访3个月后均未复发。     

儿童肺炎支原体肺炎合并Stevens-Johnson综合征

报告1例肺炎支原体肺炎合并Stevens-Johnson综合征（SJS）。患儿男,6岁。因发热、咳嗽3 d入院。胸部X射线检查提示左中下肺野炎性病变。血清肺炎支原体抗体（MP-IgM）阴性,考虑肺炎,予静脉滴注头孢地嗪联合口服阿奇霉素抗感染,静脉滴注甲泼尼龙抗炎治疗。治疗第5天患儿体温正常,第6天咳嗽好转,肺部炎症吸收,复查MP-IgM示1∶320(<1∶40),诊断肺炎支原体肺炎。治疗第7天,患儿出现一过性低热及斑丘疹。皮肤科检查：口唇肿胀,口腔黏膜充血、破溃;双眼结膜充血伴大量分泌物;手足皮肤散在疱疹及靶形红斑。诊断：肺炎支原体肺炎合并SJS。入院第9天予静脉滴注静脉注射用人免疫球蛋白联合甲泼尼龙治疗后,患儿皮肤及黏膜损害改善。治疗第12天,复查MP-IgM示1∶1 280,患儿好转出院。随访至今未复发。     

无皮疹型Stevens-Johnson综合征四例

目的报道4例无皮疹型Stevens-Johnson综合征，并对该病有关文献进行综述。方法对4例患者的发病因素、临床特征及治疗进行较为系统地观察。结果4例均为儿童，表现有发热，口唇黏膜肿胀、糜烂、出血性渗出和坏死，3例有结膜炎症，其中2例出现睑结膜纤维素样渗出；4例都无明显皮肤损害。3例进行了肺炎支原体抗体和冷凝集试验检测，肺炎支原体抗体IgG全部阳性．冷凝集试验仅l例阳性，为1：128（正常低于1：32）。2例入院前诊断“化脓性扁桃体炎”．其中l例发疹时抗链球菌溶血素O试验1240IU／mL阳性（正常低于200IU／mL）。2例柯萨奇病毒IgM检测阳性。单用抗生素治疗无效．对糖皮质激素治疗敏感，4例均痊愈。结论无皮疹型Stevens—Johnson综合征的预后较好，其病因仍以感染为主，尤其是肺炎支原体、病毒感染更应加以重视。     

白塞病误诊一例

临床资料 患者,男,37岁。因手足红斑、水疱反复发作4年,加重伴口腔、生殖器溃疡5天入院。4年前患者双手掌、足跖无明显诱因出现散发红斑、水疱,部分呈靶形损害,伴轻度痒痛感,曾于外院以＂多形红斑、巨细胞病毒感染＂口服药物治疗（具体不详）,皮疹渐消退。此后几年间患者病情反复发作4次,均表现为手足红斑、水疱,部分呈靶形损害,口服药物（自诉含有糖皮质激素,具体用法用量不详）治疗后好转,病程7～10天。     

副肿瘤性天疱疮6例临床分析

目的 探讨副肿瘤性天疱疮的临床特点、治疗与转归。方法 回顾性分析本院诊治的6例副肿瘤性天疱疮患者的临床表现、组织病理学和免疫学特点及合并肿瘤情况、治疗方法与转归。结果 所有患者均有黏膜损害，以口腔黏膜损害最突出，其中5例以口腔黏膜损害为首发症状，皮损以水疱、表皮剥脱、多形红斑、扁平苔藓样皮损常见，主要特征性的组织病理学表现为表皮内棘层松解和角质形成细胞坏死，其次为苔藓样皮炎病理征象。6例患者均合并肿瘤。6例患者除予以肿瘤针对性治疗外，均予以糖皮质激素治疗，其中4例同时联合大剂量静脉用免疫球蛋白(IVIG)治疗，最终5例患者死亡，死因分别为呼吸衰竭、肺部感染、肿瘤恶化等。结论 副肿瘤性天疱疮发病机制复杂，临床皮损多样，预后差，及早诊断并同时针对肿瘤及副肿瘤天疱疮治疗，对预后做好适当管理，可提高其生存率。     

重症多形红斑和Stevens-Johnson综合征

多形红斑（erythema multiforme,EM）患者具有同心圆形状的“靶形”皮损,但无黏膜损害和发热等系统症状。Stevens-Johnson综合征（Stevens-Johnson syndrome,SJS）患者不仅有皮肤的“靶形”损害,还有黏膜受累和系统症状,曾称为重症多形红斑（erythema multiforme majus,EMM）、重型多形渗出性红斑（erythema exsudativum multiforme majus）。一些学者认为EMM和SJS为同一种病,也有学者认为EMM和SJS不是一种病,SJS多由药物引起,可进展为中毒性表皮坏死松解症（TEN）,而EMM是EM的重型,不会进展为TEN,二者的病因、发病机制、临床表现和治疗均不同［1-3］……     

药源性重症多形红斑18例临床分析

目的:总结分析药源性重症多形红斑的临床资料、治疗及转归。方法:回顾性分析18例药物所致的重症多形红斑患者的一般资料、临床表现、实验室及辅助检查、治疗及转归。结果:致病药物以别嘌醇和卡马西平多见。患者除有皮肤损害外,还伴有黏膜损害,50%的患者有肝、肾受累。18例患者均采用足量糖皮质激素治疗,4例患者给予大剂量静脉注射用人免疫球蛋白治疗。除2例患者因肾衰及消化道出血致死外,其余患者均治愈。结论:及早采用中至大剂量糖皮质激素对促进皮损愈合、缩短病程有帮助,对病情难以控制的患者可早期应用大剂量静脉注射用人免疫球蛋白治疗。     

我对多形红斑的看法

多形红斑是一种侵犯皮肤、粘膜的急性自限性疾病,可以是特发性的,并有周期性复发倾向,也可以是对某些感染或药物的过敏反应。临床和组织学上均有多形性表现。临床上多形红斑分为轻、中、重度。轻症多形红斑表现为红斑、丘疹、小疱和大疱,常呈虹膜样或靶样损害,皮肤及粘膜损害局限,全身症状轻微或无,皮疹的过程一般为2～4周。重症多形红斑常突然起病,伴有高热、衰竭、广泛皮损,临床包括Stevens—John$on病和Lyell中毒性表皮坏死松解症(TEN)两型,死亡率高,需要及早     

7例副肿瘤性天疱疮临床分析

目的明确副肿瘤性天疱疮的I临床特点、治疗方法及预后。方法回顾分析我院2008年到2013年间收治的副肿瘤性天疱疮患者7例,分析其临床表现、伴随的肿瘤及组织病理学特点和实验室检查、治疗方法及预后。结果所有患者均有严重的黏膜损害,以口腔黏膜损害为突出表现,皮损以多形红斑、扁平苔藓样皮损和大疱最常见,最常见的特征性组织病理学为界面皮炎和凋亡的角质形成细胞。7例患者均为内脏单发肿瘤,其中以Castleman病最常见（n=6）,胸腺瘤（n=1）。其中3例死亡,1例手术后死于肺部感染,1例因自行停药而死亡,1例手术前死于败血症和严重复杂的多部位感染。手术后存活4例,最长已达5年以上,出现阻塞性细支气管炎仍未完全缓解;1例于手术后2年痊愈停药;另2例仍在1：7服泼尼松治疗。结论Castleman病是该组患者最常见的伴发肿瘤,及早发现并切除肿瘤是关键措施,手术前后的正确处理可提高其生存率。     

氨苯砜超敏反应综合征5例临床分析

目的探讨氨苯砜超敏反应综合征的临床特征和治疗方法。方法回顾分析5例氨苯砜超敏反应综合征临床资料。结果5例氨苯砜超敏综合征用药潜伏期较长,为20～42天,均以发热或皮疹为首发症状,皮疹呈多形性,为麻疹样型、多形红斑型和红皮病型,所有患者均有浅表淋巴结肿大,肝肿大,1例脾肿大,并伴有血液学异常和肝功能受损,治疗时间平均58天,4例痊愈出院,1例死于肺部感染。结论氨苯砜超敏综合征表现为发热、皮疹、黄疸、淋巴结肿大、肝损害和溶血性贫血。根据DDS用药史,排除微生物感染和其他发疹性疾病可诊断本病,治疗应遵循重症药疹治疗原则。     

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.     

Erythema multiforme due to Mycoplasma pneumoniae infection in two children

Mycoplasma pneumoniae is an important and highly relevant cause of bullous erythema multiforme, isolated mucositis, and Stevens-Johnson syndrome in children. In this article, we present two children with respiratory Mycoplasma pneumoniae infection and associated cutaneous findings within the spectrum of erythema multiforme. We review the literature associating these three entities with Mycoplasma pneumoniae infection and discuss controversies regarding the classification of erythema multiforme, as well as update reported infectious causes of the bullous form. Many understand the erythema multiforme spectrum to include bullous erythema multiforme, mucositis, and Stevens-Johnson syndrome in the order of increasing severity. We feel that this relationship should be reconsidered to help better understand the prognosis and outcomes. It is our opinion that bullous erythema multiforme is a separate, yet related condition that can occur in the context of Mycoplasma pneumoniae infection. With many similarities to mucositis and Stevens-Johnson syndrome, bullous erythema multiforme can be considered part of a spectrum of disease that includes Stevens-Johnson syndrome. Unlike mucositis and Stevens-Johnson syndrome, bullous erythema multiforme caused by Mycoplasma pneumoniae infection has low morbidity for the child. Mycoplasma pneumoniae-associated mucositis and Stevens-Johnson syndrome seem to occur along a spectrum with separate prognosis and potential pathogenesis compared with bullous erythema multiforme. Making the distinction between these conditions is valuable for predicting the child's prognosis. Patients who develop symptoms consistent with these conditions should be appropriately evaluated for Mycoplasma pneumoniae infection and closely monitored.     

Erythema multiforme associated with cytomegalovirus infection in nonimmunosuppressed patients.

BACKGROUND: It is widely known that cytomegalovirus (CMV) primarily brings about subclinical and asymptomatic infection in the early stages of life and can cause various dermatological and systemic disorders under immunosuppressed conditions. Nonimmunosuppressed individuals very rarely present with cutaneous CMV involvement. OBJECTIVE: In the present study, we described the clinical characteristics of 5 nonimmunosuppressed adult patients with positive IgM antibody to CMV. METHODS: The systemic symptoms and dermatological features of these 5 patients were described. Laboratory examinations including blood cell counts, liver and renal functions were performed. IgG and IgM antibodies to CMV were also examined at the first consultation and 2-3 months after the skin eruption. Polymerase chain reaction for CMV DNA was performed in the skin samples of the patients. RESULTS: All 5 patients had fever and complained of a sore throat. Multiple exudative erythema and target lesions with itching were observed mainly on the extremities. These symptoms and eruptions disappeared within 1 week after the onset and IgM antibody titers significantly decreased after 2-3 months. IgG antibody to CMV was already positive in 3 cases but was negative in 2 cases at the initial consultation. CONCLUSION: We propose that CMV infection may cause erythema multiforme by primary, recurrent infections or reactivation of CMV even in nonimmunosuppressed adults.     

Targetoid spongiotic reaction pattern: A case series of seven paediatric patients

We present seven cases of a targetoid eruption, clinically mimicking erythema multiforme, occurring in paediatric patients aged 12 months to 14 years. All patients presented with a pruritic targetoid eruption on body and acral sites which spared mucosal areas. All patients demonstrated a spongiotic reaction pattern on histology without lichenoid change and demonstrated excellent responses to either oral prednisolone or topical corticosteroids. We propose the term 'targetoid spongiotic reaction pattern (TSRP)' for our subset of paediatric patients. We review the literature regarding targetoid eruptions in the paediatric population.     

Erythema multiforme

In summary, the diagnosis of erythema multiforme is appropriate for a self-limiting or episodic cutaneous or mucocutaneous illness with skin lesions morphologically and histologically compatible. With typical erythema multiforme minor, characterized by classic skin lesions with or without oral erosions, most patients' disease is associated with recurrent herpes simplex infections. This is particularly true with recurrent erythema multiforme. Symptomatic conservative care, antibiotic treatment for purulent secondarily infected oral lesions, and avoidance of systemic steroids are appropriate therapeutic guidelines. The more serious syndrome, erythema multiforme major, or Stevens-Johnson syndrome, is characterized by skin lesions that are somewhat atypical and different from those of erythema multiforme minor in association with erosions on multiple mucosal surfaces. Drugs and mycoplasmal infections are important precipitating factors for erythema multiforme major. Hospitalization and laboratory tests are often required because of the severity of the illness and the occasional damage to other organ systems. Conservative, symptomatic care, withdrawal of any drug that may have caused the illness, treatment of any mycoplasmal infection, and antibiotic therapy for purulent secondarily infected lesions are worthwhile therapeutic measures. Early treatment with systemic steroids may be helpful in preventing further damage, and the risks and potential benefits of such therapy must be evaluated on an individual basis.     

Approach to the hospitalized patient with targetoid lesions

Approaching the hospitalized patient with skin disease can be daunting. This article focuses on a practical approach to the patient with targetoid lesions. The discussion focuses on differentiating erythema multiforme from Stevens-Johnson syndrome and toxic epidermal necrolysis. In addition, the article offers a concise review of the broader differential diagnosis of targetoid lesions including ecthyma gangrenosum, fixed drug eruption, erythema multiforme-like drug reaction, vasculitis, acute hemorrhagic edema of infancy, erythema chronicum migrans, connective tissue diseases, and blistering diseases.     

Recurrent and continuous erythema multiforme– a clinical and immunological study

A series of 26 patients with recurrent erythema multiforme was studied. A distinct clinical and immunological subgroup was found called ‘continuous erythema multiforme’ characterized by the presence of atypical lesions in addition to typical lesions, both of which occur continuously and a high level of circulating immune complexes with low levels of haemolytic complement. Herpes labialis preceded erythema multiforme in 17 of the 26 patients (65%) but in no cases could live virus be isolated from the lesions of erythema multiforme. Circulating immune complexes were found in 50 of 129 sera, being found in only 18 % sera between attacks and more commonly in the first 24 h of erythema multiforme lesions (58%). Immunological studies failed to provide conclusive evidence that erythema multiforme is solely immune complex mediated.     

Mucositis Secondary to Chlamydia pneumoniae Infection: Expanding the Mycoplasma pneumoniae–Induced Rash and Mucositis Concept

The term Mycoplasma pneumoniae–induced rash and mucositis (MIRM) was recently proposed to identify the mucocutaneous condition secondary to M. pneumoniae infection that had historically been regarded among the more confusing pathologies of erythema multiforme and Stevens–Johnson syndrome. Based on a number of previous reports, these syndromes require differentiation since they have different prognoses and specific treatment requirements. We report a case of oral and genital erosions that strongly resembled MIRM without rash but were found to be secondary to a Chlamydia pneumoniae infection. After a thorough review of the literature on this subject, we propose that C. pneumoniae should also be considered a potential causative agent of MIRM and that this term should be amended to include C. pneumoniae infection.     

Adult linear IgA bullous dermatosis: a polymorphic disorder

We report on two patients with unusual forms of adult linear IgA bullous dermatosis. One was a middle-aged woman who had targetoid lesions and bullae on her trunk and extremities. This patient first presented with lesions that clinically resembled erythema multiforme, but these evolved into a widespread eruption with bulging, elongated bullae. Examination of a biopsy specimen showed changes compatible with dermatitis herpetiformis and bullous pemphigoid. Findings on immunofluorescence studies showed deposition of linear IgA at the basement membrane zone. The second patient was an elderly woman with intensely pruritic vesicles whom we classified as having vesicular pemphigoid, until the linear IgA band on direct immunofluorescent test results became the predominant immunofluorescent finding. These cases are reported because of their unusual clinical presentations. The mechanism for the targetoid lesions in the first patient is discussed.