葡萄球菌烫伤样皮肤综合征177例临床分析

【摘要】 目的 探讨177例葡萄球菌烫伤样皮肤综合征（SSSS）患儿的诊断和治疗。方法 回顾性研究177例SSSS患儿的临床资料。探讨其年龄和地区分布、发病诱因、临床特征、详细的实验室检查、病原菌分离培养和药敏、治疗方法以及预后情况。结果 本组共177例,发病年龄中位数约为1岁3个月23天,多见于婴幼儿期,男女性别比约为8 ∶ 9,71.2%于夏秋季节患病。患儿多来自农村,148例（83.6%）无明确的发病诱因,多起病于颜面部（71.6%）。129例检查心肌酶谱104例（80.6%）异常,142例中38.7%分离出金黄色葡萄球菌。对近3年金黄色葡萄球菌药敏试验结果分析显示,头孢菌素类和阿莫西林/棒酸的敏感率分别为95.2%和95.0%。72.9%双联抗生素治疗有效,36.2%患儿早期予以丙种球蛋白,对于心肌酶异常患儿早期给予保护心肌药物,所有患儿痊愈且不留瘢痕。结论 对于出现颜面部的急性红斑和表皮脱屑的婴幼儿应高度怀疑为SSSS,且予以及时的抗生素治疗,头孢菌素类和半合成青霉素复合制剂应是首选有效的药物,同时早期合用丙种球蛋白和保护心肌药物有利于疾病恢复。     

Irritant contact dermatitis in healthcare workers as a result of the COVID‐19 pandemic: a cross‐sectional study

COVID‐19 healthcare workers (HCWs) require frequent handwashing and use of personal protective equipment (PPE) to prevent infection. However, evidence is emerging that these practices are causing adverse effects on their skin integrity. A single‐centre, cross‐sectional study of HCWs from an Irish hospital was undertaken to evaluate the degree of COVID‐19‐related irritant contact dermatitis (ICD) between April and May 2020. Of 270 participants surveyed, 223 (82.6%) reported symptoms of ICD. The hands were the most commonly affected site (76.47%) and the most frequently reported symptom was dry skin (75.37%). Nearly all (268; 99.26%) HCWs had increased hand‐washing frequency, but 122 (45.35%) did not use emollients. In the ICD group, 24.7% cited a history of dermatitis compared with 4.3% of unaffected staff (P < 0.001). The ICD group recorded PPE usage for an average of 3.15 h compared with the non‐ICD group at 1.97 h (P = 0.21). Promoting awareness of COVID‐19‐related ICD is vital to highlight prevention and treatment for frontline staff.     

Antibiotic sensitivity and clinical outcomes in staphylococcal scalded skin syndrome

Staphylococcal scalded skin syndrome causes widespread skin denudation primarily in infants < 1 year old. Selection of empiric therapy is complicated by rising rates of antibiotic resistance in community-acquired staphylococcal infections. Consistent with a previous study, this retrospective review found that SSSS-associated isolates were more likely to be clindamycin-resistant and less likely to be methicillin-resistant compared to overall staphylococcal infections. We favor cephalosporins and penicillinase-resistant penicillins (eg, oxacillin) for empiric management of SSSS, with consideration of adding MRSA coverage in communities with high MRSA prevalence or failure to improve following several days of treatment.     

Prolonged cyclosporine treatment of severe or recalcitrant psoriasis: descriptive study in a series of 20 patients

Background  Although long‐term cyclosporine administration may induce toxic effects, it may be the only option for the treatment of severe psoriasis. The objective of the present study was to retrospectively evaluate efficacy and safety of long‐term cyclosporine treatment in a cohort of patients affected with moderate to severe psoriasis, recalcitrant or unresponsive to other treatments. Possible risk factors predicting an intolerance to cyclosporine were also investigated. Materials and methods  Data were collected on psoriatic patients treated with cyclosporine for at least six months at our Psoriasis Outpatient Unit between January 2005 and September 2010. The primary measure for clinical efficacy was the PASI 75 response. Cyclosporine safety was assessed through the review of both laboratory tests and the adverse events registered during the treatment. Results  Twenty patients affected with plaque or erythrodermic psoriasis were evaluated. At Week 16, the PASI 75 response was achieved by 85% of patients. Adverse events occurred in eight patients (40%): four experienced an increase in serum creatinine levels to more than 30% of their pre‐treatment values and four developed hypertension. Among these patients, five discontinued cyclosporine. Side effects resolved after stopping treatment. Conclusions  Our findings suggest that long‐term cyclosporine regimen can be justified in severe psoriasis not responsive to other treatments. When cyclosporine administration is required, obesity, pre‐treatment controlled hypertension, increased age (>70 years), and metabolic syndrome should be taken into consideration, as a significant correlation with occurrence of cyclosporine‐induced side effects has been found.     

葡萄球菌烫伤样皮肤综合征208例临床分析

目的回顾分析208例葡萄球菌烫伤样皮肤综合征患儿的临床资料,对部分病原菌进行表皮剥脱毒素及药敏检测,了解本地区致病菌分泌表皮剥脱毒素血清型别及药敏情况。方法记录所有病例的临床资料,并对其中30株金黄色葡萄球菌(以下简称SA)应用反向乳胶凝集法检测表皮剥脱毒素,对51株SA应用琼脂稀释法进行了14种抗生素的药敏试验。结果 30株SA中,21株表皮剥脱毒素A,B均阳性,8株单纯表皮剥脱毒素B阳性。51株SA药敏青霉素耐药率为96.08%,对红霉素耐药率为82.35%。对苯唑青霉素、头孢类抗生素、莫匹罗星、万古霉素和夫西地酸均未发现耐药菌。结论引起本病的SA以表皮剥脱毒素A,B均阳性的菌株为主。青霉素和红霉素耐药率较高,不适合治疗本病。头孢菌素、万古霉素和夫西地酸可作为可选择的抗生素。     

Retrospective analysis of 12 Korean patients with paraneoplastic pemphigus

Paraneoplastic pemphigus (PNP) is a rare, life‐threatening, autoimmune, mucocutaneous blistering disease associated with neoplasia. Both humoral and cellular immunity are involved in the pathogenesis of PNP. Characteristically, PNP has a diverse spectrum of clinical and immunopathological features. We retrospectively analyzed 12 Korean patients with PNP who were diagnosed between 1993 and 2011. We performed analysis of the clinical features, clinical outcomes, underlying neoplasia, histological features and laboratory findings. All of the patients except one had severe mucosal involvement. Two patients had only mucosal lesions but no cutaneous involvement was observed. Erythema multiforme or lichen planus‐like eruptions rather than bullous lesions were more commonly observed skin rashes. The most common histological features were interface dermatitis and apoptotic keratinocytes. There were associated hematological‐related neoplasms in 11 patients, with Castleman's disease (n = 4) as the most frequent. Twelve patients were followed for 5–148 months (mean, 43.0). The prognosis depended on the nature of the underlying neoplasm. Six patients died due to respiratory failure (n = 3), postoperative septicemia (n = 1), lymphoma (n = 1) and sarcomatosis (n = 1). The 2‐year survival rate was 50.0%, and the median survival period after diagnosis was 21.0 months. Immunoblotting was performed in 12 patients and autoantibodies to plakins were detected in 11 patients. The results of this study demonstrated the clinical, histological and immunological diversity of PNP. Widely accepted diagnostic criteria that account for the diversity of PNP are needed.     

Outcome measures for vulval skin conditions: a systematic review of randomized controlled trials

Symptoms and signs of vulval skin disorders are common. These conditions can have a considerable impact on quality of life, restricting physical activities and causing difficulty in everyday activities and may also affect social, psychosexual and psychological well‐being. There are no standardized measures routinely used to assess the impact of vulval disease on daily life. To report outcome measures used in clinically based randomized controlled trials (RCTs) investigating therapeutic interventions in vulval disease. The Medline, EMBASE and CENTRAL databases were searched to identify RCTs of vulval skin conditions written in English. Studies with laboratory tests or survival rates as the primary outcome, or those investigating menopausal symptoms or infections were excluded. Twenty‐eight published RCTs were included. The vulval conditions represented were vulvodynia (n = 14), lichen sclerosus (n = 9), vulval intraepithelial neoplasia (n = 2), vulval pruritus (n = 2) and lichen planus (n = 1). The 28 RCTs measured 25 different outcomes, using 49 different scales. The method of outcome assessment was lacking on nine occasions. Only 21% (six of 28) of included trials had a clearly stated primary outcome. Patient‐reported outcomes were more commonly reported than clinician‐related outcome measures. The most commonly reported patient‐rated outcome measure was a reduction in pain (measured 15 times) and an overall improvement in symptoms using a patient global assessment (measured 11 times). The most commonly reported clinician‐rated outcome was an overall assessment of the appearance of affected sites (measured 13 times). There were no agreed standard scales used for the global assessments. Only nine of the recorded outcome measure tools were designed to assess vulval disease or sexual functioning, the remainder were general measures. There is heterogeneity in the outcome measures used when reporting therapeutic interventions in vulval disease. This field of dermatology would benefit from development of a vulval‐specific outcome measure and the establishment of a core outcome measure set.     

Capillary‐Venous Malformation in the Lower Limb

Regional capillary malformation of a lower extremity is associated with the overgrowth of bone or soft tissue in several disorders, most commonly Klippel–Trenaunay syndrome and Parkes Weber syndrome. We have observed a subset of patients with a capillary malformation of the leg, minor growth disturbance, and prominent veins. The objective of the current study is to describe a series of patients with regional capillary malformation of the lower extremity in association with phlebectasia. This is a retrospective series of 17 patients diagnosed with capillary‐venous malformation of the lower extremity. We excluded patients with clinical or radiographic evidence of lymphatic or arteriovenous malformation. Age, presentation, associated features, radiographic findings, and management were documented. In most patients the capillary malformation covered a large area without sharply demarcated borders. Four patients had one or more discrete, well‐defined capillary stains involving less than 5% of the total surface area of the affected lower limb. Prominent veins were most common in the popliteal fossa and on the knee and dorsal foot. Approximately two‐thirds of patients had a leg length discrepancy, with the affected leg being longer (n = 6) or shorter (n = 4); in many the affected leg was also slightly larger (n = 8) or smaller (n = 4) in girth. Radiographic imaging showed dilatation of superficial (n = 16), muscular (n = 9), and deep veins (n = 6). We characterize a subset of patients with regional capillary‐venous malformation of the lower extremity with prominent veins and minor hypotrophy/hypertrophy that differs from Klippel–Trenaunay syndrome (capillary‐lymphatic‐venous malformation) but belongs at the minor end of the spectrum of vascular disorders with overgrowth.     

Trigeminal trophic syndrome: Report of a case and review of the published work

Trigeminal trophic syndrome is a rare complication of trigeminal nerve injury that causes facial ulceration, anesthesia and paresthesia in the same trigeminal dermatomes. We present a case of a 65‐year‐old woman with a history of meningioma resection 18 years prior who presented 16 years later with an intractable ulceration around her left nasolabial sulcus. Pain and light‐touch sensations around the ulcer were decreased. She admitted to frequent manipulation due to a crawling sensation. A skin biopsy showed acanthotic changes and a decreased number of peripheral nerve fibers. Trigeminal trophic syndrome was diagnosed. Carbamazepine was not effective, and the ulcer persisted at 7 months after the initial presentation. We reviewed 36 English‐language publications from 2003 to 2012, and analyzed 61 cases of trigeminal trophic syndrome, including this patient. The mean age was 53.3 ± 19.7 years (range, 6–91). The right side of the face was more commonly affected (57%) than the left side. The ala nasi were involved in 48 cases (79%), followed by the cheek in 17 cases (28%). A corneal lesion was observed in 11 cases (18%), suggesting the importance of ophthalmologic consultations. The two major etiologies were trigeminal nerve ablation (18 cases; 30%) and cerebrovascular accidents (18 cases; 30%). The latent period ranged from days to 30 years. Gabapentin and carbamazepine were frequently administrated with variable efficacy. Application of thermoplastic dressings or negative pressure wound therapy demonstrated favorable outcomes. Surgery was an option with a high recurrence rate. Trigeminal trophic syndrome remains a clinical challenge.     

The pharmacokinetics of tacrolimus after first and repeated dosing with 0.03% ointment in infants with atopic dermatitis*

Background In adults and children aged > 2 years, systemic absorption of tacrolimus from tacrolimus ointment is very low. In this study, the pharmacokinetics of tacrolimus 0.03% ointment were investigated in infants aged 3–24 months. Methods The pharmacokinetics of tacrolimus after first and repeated topical application of tacrolimus 0.03% ointment were evaluated in 53 infants (age, 3–24 month) with atopic dermatitis requiring treatment with mid‐potency topical corticosteroids. Patients were grouped according to percentage of body surface area affected (Group 1: 5–20%; Group 2: > 20–40%; Group 3: > 40%). After stratification, patients were randomized (double‐blind) to receive once‐daily or twice‐daily tacrolimus 0.03% ointment. Results Blood samples taken on days 1 and 14 (first and last application) showed minimal systemic tacrolimus exposure. Overall, 97% of blood samples assayed contained tacrolimus concentrations < 1 ng/ml, and 20% were below the lower limit of quantification (0.025 ng/ml). Systemic tacrolimus exposure was variable, but tended to increase as the treated body surface area increased. Mean apparent half‐life of tacrolimus was 80 ± 35 h (range: 25–175 h). Most patients experienced substantial clinical improvement in their atopic dermatitis. There were no clinically significant changes in laboratory values, and the most frequently reported adverse events were minor infections and local skin irritations. Conclusions Tacrolimus 0.03% ointment in infants is associated with very low systemic exposure to tacrolimus. Treatment was well tolerated and led to considerable clinical improvement.     

Temporal Trends in Mucocutaneous Findings Among Human Immunodeficiency Virus 1‐Infected Children in a Population‐Based Cohort

The objective of the study was to determine the prevalence of pediatric human immunodeficiency virus 1 (HIV‐1) mucocutaneous manifestations in the era of highly active antiretroviral therapy (HAART). We conducted population‐based, prospective, multicenter pediatric HIV‐1 surveillance in 276 children with perinatally acquired HIV‐1 from 1988 to 2009. Centers for Disease Control and Prevention (CDC)‐defined HIV‐1 related mucocutaneous conditions among the 276 children were: category A (n = 152), B (n = 60), and C (n = 1). Nearly half of the category A and B diagnoses (43.4% [66/152] and 35.0% [21/60], respectively) occurred in the first year of life, with 59.2% (90/152) and 61.7% (37/60), respectively, occurring in the first 2 years of life. The most frequent infectious diagnosis was oropharyngeal thrush (n = 117, 42.4%); the most common inflammatory diagnosis was diaper dermatitis (n = 71, 25.7%). There was a temporal decline in the prevalence of A (pre‐HAART cohort, 123; post‐HAART cohort, 29; p < 0.01) and B (pre‐HAART, 55; post‐HAART, 5; p < 0.01) mucocutaneous diagnoses. In children with perinatal HIV‐1, there was a significant decline in CDC category A and B mucocutaneous diagnoses by temporal cohort, consistent with the introduction of antiretroviral medications and HAART. Clinical category A and B mucocutaneous diagnoses were most common in the first 2 years of life, emphasizing the importance of early HIV‐1 testing and HAART initiation.     

Ocular involvement in pemphigus vulgaris

Pemphigus vulgaris (PV) is an autoimmune disorder affecting the skin and mucous membranes. Ocular involvement in PV has been reported but its prevalence and clinical characteristics are not well defined. This prospective cross‐sectional study of 103 PV patients was designed to determine the prevalence, clinical types and epidemiological trends of ocular involvement in a population of Iranian patients with PV. Ocular involvement was present in 17 (16.5%) patients. Conjunctivitis was the most prevalent type of ocular involvement (9/17, 52.9%), followed by erosion of the palpebral conjunctiva (7/17, 41.2%). Erosion of the bulbar conjunctiva was noted in only one patient (5.9%). The most commonly reported symptoms were eye irritation (76.5%) and redness (76.5%). No significant relation was found between ocular involvement and disease activity (partial remission or relapse). Mucoid discharge was significantly more common in patients with conjunctival erosions as compared to patients with conjunctivitis (P = 0.038). We conclude that ocular involvement is not rare in PV; 16.5% of PV patients develop ocular disease independent of the disease activity and extension. Conjunctivitis is the most common type of involvement, however, palpebral conjunctival erosion is more frequent than previously realized.     

Allergic contact dermatitis in children; has there been a change in allergens?

Allergic contact dermatitis (ACD) is now recognized as a common relevant diagnosis in children. We reviewed the results of patch tests in children under 16 years of age over the 3‐year period January 2009 to December 2011, comparing the results with those in a similar 3‐year retrospective study performed in our unit for the period August 1999 to September 2002. In total, there were 137 children (70 girls and 67 boys) aged 3–15 years enrolled. Of these, 28 girls (40%) and 22 boys (32.8%) had a positive reaction to at least one allergen, with nickel sulfate and potassium dichromate being the most common allergens, each having an incidence of 7.2%, in contrast to the 20% incidence of nickel allergy in 1999–2002. The rate of fragrance allergy was unchanged at 5.8%, and rubber allergy incidence was greatly reduced. These results show that allergens may be changing in children, and that patch testing is still a useful test to consider.     

Infection in autoimmune bullous diseases: A retrospective comparative study

Certain autoimmune bullous diseases (AIBD), including pemphigoid and pemphigus, confer increased infection risk. Infections have not been systematically studied in these conditions, however. Little is known about infection risk in these conditions, particularly dermatitis herpetiformis (DH). We aimed to characterize and compare infection patterns and risk factors in patients with pemphigoid, pemphigus, and DH. We retrospectively studied the medical records of Olmsted County, Minnesota, residents who had a diagnosis of AIBD between 1 January 1998 and 1 January 2011. Of 81 patients studied, 54 (67%) had pemphigoid, 11 (14%) had pemphigus and 16 (20%) had DH. Most patients studied developed at least one localized infection (72%) or one systemic infection (83%). Almost one‐third of patients (31%) developed infections requiring hospitalization or contributing to death. All patients taking systemic corticosteroids experienced a localized or systemic infection during the follow‐up period. Systemic infections were significantly less frequent in patients with DH than those with pemphigoid or pemphigus (P = 0.03), as were systemic infections requiring hospitalization or contributing to death (P = 0.002). Patients with DH were significantly less likely to require systemic corticosteroids (P < 0.001) and significantly more likely to receive dapsone (P = 0.002). The study design was retrospective and a limited number of patients met the inclusion criteria. Patients with AIBD frequently developed localized and systemic infections, a substantial portion of which contributed to hospitalization or death. Patients with DH experienced infections of lesser severity than patients with pemphigoid or pemphigus.     

Overview of Dermatologic Disorders of Neonates in a Central Regional Intensive Care Unit in Hungary

The immaturity and vulnerability of the skin and epidermal barrier function and the frequent iatrogenic complications following diagnostic and therapeutic procedures are often associated with skin manifestations in infants in neonatal intensive care units (NICUs). The aim of the current study was to investigate dermatologic disorders in neonates in our NICU. A prospective cohort study was conducted in the NICU at the Department of Pediatrics at the University of Szeged between January 2012 and January 2013. All full‐ and preterm infants hospitalized in the NICU underwent whole‐body skin examinations and all dermatologic disorders and treatment modalities were recorded. Eighty‐nine dermatologic conditions were detected in 64 of the 211 neonates admitted to the NICU. A wide variety of clinical symptoms accompanied these conditions in these preterm and severely ill full‐term infants. A considerable proportion of the disorders that were seen resulted from the immaturity of the skin and various iatrogenic complications. Dermatologic disorders are frequent in neonates requiring intensive care. Prevention, early detection, and optimal treatment of these disorders with modern, standardized skin care management strategies can result in significant improvements in barrier function and in the integrity of the skin, increasing the overall efficacy of neonatal intensive care.     

Analytical study of pustular eruptions in neonates

Pustular eruptions are commonly encountered in neonatal practice. Much confusion exists among clinicians because of the similarity in clinical lesions, paucity of relevant literature, and varied nomenclature used for these diseases. This often results in inappropriate diagnoses and therapies, besides subjecting the neonates to invasive and traumatic investigative procedures. We conducted a comprehensive study of pustular eruptions in 100 neonates, using the clinical examination and simple laboratory tests to arrive at a practical diagnostic and therapeutic approach to this problem. Of the 100 neonates with pustular eruptions, 36% were in the early neonatal period (first week of life). A slight male preponderance with a male:female ratio of 1:0.79 was observed. The majority of the families of these infants had poor socioeconomic status (96%) and were living in slums (71%). A study of their educational status revealed that 54% of the mothers were uneducated. Fifty-seven percent of the neonates were born at home. The clinical pattern of diseases among these neonates was that 58% of them had infections [impetigo (23%), intertrigo (14%), scabies (6%), and viral diseases (6%)]. Noninfectious diseases (42%) included miliaria pustulosa, erythema toxicum neonatorum, epidermolysis bullosa, and contact dermatitis. Simple laboratory investigations helpful in establishing the diagnosis were smears processed with Gram (24%) and Giemsa (39%) stains and wet mounts with 10% potassium hydroxide (KOH) solution (27%) for direct microscopic examination. More than half (53%) of the patients required no specific treatment except for counseling and medications to alleviate symptoms, while others with an infectious etiology responded to topical and or systemic antibiotics/antifungals. Pustular eruptions in neonates include both infectious and noninfectious diseases. Simple laboratory tests such as Gram- and Giemsa-stained smears, direct microscopy with 10% KOH wet mounts, bacterial and fungal cultures are helpful in establishing the diagnosis and occasionally skin biopsy is needed. A practical diagnostic and therapeutic approach to this problem is discussed.     

儿童葡萄球菌性烫伤样皮肤综合征临床及实验室特点分析

目的:分析葡萄球菌性烫伤样皮肤综合征(staphylococcal scalded skin syndrome, SSSS)患儿的临床及实验室特点。方法:分析2014年5月-2017年5月共49例SSSS患儿的临床特点,并以其为观察组,同期选取60例健康体检儿童为健康对照组,比较两组儿童T细胞亚群及免疫球蛋白结果。对患儿脓疱液或脓性分泌物进行细菌培养,将分离到的金黄色葡萄球菌进行药敏分析。结果:42例SSSS患儿皮损初发于面部。观察组患儿T细胞亚群CD3^+、CD4^+、免疫球蛋白IgG水平明显低于健康对照组,差异有统计学意义(P<0.05)。共分离得到28株细菌,以金黄色葡萄球菌为主,占71.43%(20/28)。结论:SSSS主要引起5岁以下婴幼儿感染,患儿T淋巴细胞亚群及免疫球蛋白指标的检测为了解患儿的免疫功能状态、免疫调节及支持治疗提供了理论依据。同时,病原学依据对该疾病的精准治疗、预后评估具有重要意义。     

Prevalence and Characterization of Pruritus in Epidermolysis Bullosa

Qualitative data suggest that pruritus is a burdensome symptom in patients with epidermolysis bullosa (EB), but the prevalence of pruritus in children and adults with EB and factors that contribute to pruritus are unknown. The objective of the current study was to quantitatively identify and to characterize pruritus that EB patients experience using a comprehensive online questionnaire. A questionnaire was developed to evaluate pruritus in all ages and all types of EB. Questions that characterize pruritus were included and factors that aggravate symptoms were investigated. Patients from seven North American EB centers were invited to participate. One hundred forty‐six of 216 questionnaires were completed (response rate 68%; 73 male, 73 female; median age 20.0 years). Using a 5‐point Likert scale (1 = never, 2 = rarely, 3 = sometimes, 4 = often, 5 = always), itchiness was the most bothersome EB complication (mean 3.3). The average daily frequency of pruritus increased with self‐reported EB severity. Pruritus was most frequent at bedtime (mean 3.8) and interfered with sleep. Factors that aggravated pruritus included healing wounds, dry skin, infected wounds, stress, heat, dryness, and humidity. Pruritus is common in individuals with EB and can be bothersome. Future studies will need to investigate the most effective treatments given to individuals with EB for pruritus.