一般型皮肤异色病样淀粉样变11例分析

目的:分析一般型皮肤异色病样淀粉样变(PLCA)的临床特点。方法:收集总结我院11例经组织病理确诊的一般型皮肤异色病样淀粉样变患者的临床资料(病史、皮损特点、治疗及转归等)进行分析。结果:11例均为成人期发病。首发部位均在四肢,9例日晒后加重,7例伴有苔藓样丘疹,3例伴有水疱,3例有家族史。结论:在临床中,表现为皮肤异色、苔藓样丘疹,首发于四肢,无智力低下和身材矮小,需考虑一般型PLCA的可能。     

老年重症药疹33例临床分析

目的探讨老年重症药疹患者的临床特点。方法对本院2000年1月～2007年12月收治的33例老年重症药疹患者的临床资料进行回顾性分析。结果重症多形红斑型药疹所占比例最高,为48.48%;重症药疹中致敏药物以别嘌呤醇占首位,为32.26%;伴有基础疾病患者的皮疹控制时间及住院天数均较无基础疾病患者长(t值分别为2.377,2.160,P均<0.05),有肝功损害的患者糖皮质激素起始用量大于无肝功能损害的患者(t=2.278,P<0.05)。结论糖皮质激素起始治疗量与重症药疹类型、是否伴有肝脏损害关系密切;皮疹的控制时间及住院天数与是否合并基础疾病有关。     

老年性皮肤病

20100670老年重症药疹33例临床分析/罗婧莹(桂林医学院附院皮肤科),陈德华,黄熙…∥中国皮肤性病学杂志.-2009,23(2).-90～91对本院2000年1月～2007年12月收治的33例老年重症药疹患者的临床资料进行回顾性分析。结果重症多形红斑型药疹所占比例最高,为48.48%;重症药疹中致敏药物以别嘌呤醇占首位,为32.26%;伴有基础疾病患者的皮疹控制时间及住院天数均较无基础疾病患者长(t值分别为2.377,2.160,P均<0.05),有肝功损害的患者糖皮质激素起始用量大于无肝功能损害的患者(t=2.278,P<0.05)。认为糖皮质激素起始治疗量与重症药疹类型、是否伴有肝脏     

口腔寻常型天疱疮

寻常型天疱疮(PV)是一种自身免疫性皮肤黏膜疾病,常首发于口腔并逐渐累及其他部位黏膜和皮肤。许多患者,特别是初期仅表现为口腔黏膜损害的患者,常被误诊为其他疾病并影响治疗和预后。本文报告1例。     

50例婴幼儿斑秃相关因素分析

为探讨婴幼儿斑秃的相关因素与斑秃的发病及预后的关系,回顾性分析50例婴幼儿斑秃患儿的临床资料及实验室检查结果。35例(70%)患儿合并湿疹,23例(46%)甲状腺功能异常,贫血指标异常者14例(28%)。斑秃可能同时合并甲状腺疾病、贫血、白血病,如能在婴幼儿期发现并对相关疾病进行治疗,对斑秃病情的转归及探索婴幼儿斑秃是否为上述疾病的早期临床表现,将有重要的临床指导意义。     

泛发性带状疱疹患者21例外周血淋巴细胞亚群及临床分析

目的分析泛发性带状疱疹(HZG)患者外周血淋巴细胞亚群的变化及其临床特征。方法回顾性分析近年来收治的21例HZG患者的临床资料;应用流式细胞仪检测HZG患者外周血中淋巴细胞亚群的分布情况,另各选择21例本院局限型带状疱疹(HZ)患者及健康体检者作为对照组。结果 HZG组与局限型HZ组、健康对照组相比,CD3~+CD4~+T细胞百分比、CD3~+CD4~+T细胞绝对值、CD3~+T细胞绝对值、CD3~+CD4~+/CD3~+CD8~+比值明显降低,差异有统计学意义(P0.05);而外周血B细胞百分比差异无统计学意义(P0.05)。HZG患者中19.05%有肿瘤性疾病。结论 HZG患者的免疫功能低于局限型HZ患者;T淋巴细胞免疫功能下降可能是导致HZ血源性播散引起HZG的重要原因;肿瘤性疾病患者更易患HZG。     

卡波西样血管内皮瘤117例临床分析

目的总结分析卡波西样血管内皮瘤(Kaposiform hemangioendothelioma,KHE)患者的临床特点及治疗经验。方法选择并分析2008年1月-2018年12月在四川大学华西医院就诊的117例KHE患者的临床资料,结合相关文献,回顾性总结分析其临床特征、并发症、治疗及预后等特点。结果共入组117例KHE患者,男64例、女53例,比例约为1.20∶1,患者年龄1 d^5岁,平均年龄2.74个月,年龄<1岁的患者105例(89.74%)。伴有KMP(Kasabach-Merritt phenomenon,KMP)者83例,主要临床表现为皮肤损害、皮下或骨关节肌肉的浸润性肿物、伴有运动受限、急性心衰、胸腔积液、心包积液或凝血出血相关疾病等并发症。病灶直径范围2~26 cm,平均直径为9.04 cm。提示年龄、病灶大小与患者是否发生KMP有关(P均<0.05)。采用西罗莫司、泼尼松、长春新碱等药物治疗55例,手术治疗25例,联合治疗32例,未治疗行观察随访5例。转归:临床治疗有效103例(88.03%),9例(7.69%)因急性心衰、呼吸衰竭、弥散性血管内凝血等并发症死亡。结论绝大多数KHE患者在1岁以前发病,并且发生KMP风险更高。KHE临床表现主要与各种并发症有关,早期明确诊断和及时治疗对减少并发症的发生、改善预后十分重要。     

急性泛发性发疹性脓疱病11例临床回顾性分析

目的:探讨急性泛发性发疹性脓疱病(acute generalized exanthematous pustulosis, AGEP)的诊断、病因、临床特点、实验室检查及治疗。方法:应用EuroSCAR(2001修订版)作为诊断标准对2011年12月-2017年3月收治的11例AGEP住院患者进行回顾性分析。结果:11例患者中9例(81.8%)发病前有明确用药史,7例(63.6%)出现发热,10例(90.9%)中性粒细胞绝对计数升高,2例(18.2%)伴有内脏受累。皮损均表现为在弥漫性肿胀性红斑基础上密布直径5 mm的非毛囊性脓疱。AGEP病程短,有自限性,去除可能的病因,予糖皮质激素和对症支持治疗均可获得较好疗效。结论:EuroSCAR(2001)可以有助于皮肤科医师诊断AGEP。药物是AGEP发病的最主要诱因。AGEP通常有自限性,预后良好,但仍有少数患者可伴有系统受累。     

中西医结合治疗儿童过敏性紫癜74例

自 1 996年至 1 998年我科采用中西医结合方法治疗儿童过敏性紫癜 74例 ,疗效满意 ,现报告如下 :1　临床资料　74例均为住院病例 ,男 34例 ,女 40例 ,年龄最大 1 6岁 ,最小 5岁 ,平均年龄 9 6岁 ,病程 6 - 30天。春夏季发病者 8例 ,秋冬季发病者 66例。发病部位 :全身泛发者 2例 ,四肢 5例 ,两小腿 67例。本组病例均有皮肤紫癜 ,尿中有蛋白及红细胞的 33例 (44 6 % ) ,腹痛 2 6例及便血 3例 (39 2 % )。本组病例同时伴有关节、肌肉肿痛的有 43例(58 1 % )。2　治疗方法2 1　西药治疗　氟美松 5 - 7ｍｇ,红霉素 0 3 - 0 6ｇ,静脉点滴 ,每…     

别嘌醇药疹56例临床特征及预后因素分析

目的 :探讨别嘌醇药疹的临床特征及预后因素。方法 :收集2009年1月—2015年6月该科住院确诊的56例别嘌醇药疹患者的临床资料,对药疹的潜伏期、起病特点、基础疾病、皮肤黏膜损害、系统损害、实验室检查及治疗与转归进行回顾性分析,并进一步利用Logistic回归分析对其预后因素进行分析。结果:别嘌醇药疹的潜伏期平均为(24.2±13.8)d,其中重症药疹占55.4%,发病初伴有发热者占64.3%,伴有黏膜损害者占53.6%。85.7%患者血常规异常,39.3%患者诊断药物性肝损伤,66.1%患者估算肾小球滤过滤(eGFR)≤90 ml/(min·1.73m~2)。所有患者均系统使用了糖皮质激素,其最大用量(泼尼松)平均为(1.323±0.565)mg/kg·d,其中23.2%患者同时使用了丙种球蛋白静脉滴注。经住院系统治疗,7例(12.5%)患者死亡,Logistic回归分析显示,估算肾小球滤过率及既往有无药物过敏史是别嘌醇药疹发生死亡的主要危险因素。结论:别嘌醇易引起重症药疹,且死亡率较高,肾功能损害的程度及既往有无药物过敏史是影响预后的主要危险因素。     

Lupus erythematosus and other autoimmune diseases related to statin therapy: a systematic review

BACKGROUND: Statins have been increasingly associated with drug-induced autoimmune reactions, including lupus erythematosus. OBJECTIVE: To identify and determine the clinical and biological characteristics of statin-induced autoimmune reactions. MATERIAL AND METHODS: The MEDLINE database (1966 to September 2005) was used to identify all reported cases of statin-induced autoimmune diseases. The keywords used were statins, 3-hydroxy-3-methylglutaryl coenzyme A reductase inhibitors, adverse effects, autoimmune disease, lupus erythematosus, dermatomyositis and polymyositis. RESULTS: Twenty-eight cases of statin-induced autoimmune diseases have been published so far. Systemic lupus erythematosus was reported in 10 cases, subacute cutaneous lupus erythematosus in three cases, dermatomyositis and polymyositis in 14 cases and lichen planus pemphigoides in one case. Autoimmune hepatitis was observed in two patients with systemic lupus erythematosus. The mean time of exposure before disease onset was 12.8+/-18 months; range 1 month-6 years. Systemic immunosuppressive therapy was required in the majority of cases. In many patients, antinuclear antibodies were still positive many months after clinical recovery. A lethal outcome has been recorded in two patients despite aggressive immunosuppressive therapy. CONCLUSION: Long-term exposure to statins may be associated with drug-induced lupus erythematosus and other autoimmune disorders. Fatal cases have been reported despite early drug discontinuation and aggressive systemic immunosuppressive therapy.     

Pyoderma gangrenosum and underlying diseases in Japanese patients: A regional long‐term study

Pyoderma gangrenosum (PG) is a chronic inflammatory disease of unknown cause that presents as an inflammatory and ulcerative disorder of the skin. PG is often associated with an underlying systemic disease. However, the frequencies of the underlying diseases are unclear in Japanese patients. In this retrospective, observational study, all patients diagnosed with PG who visited dermatology departments of nine regional hospitals in and around Ibaraki Prefecture were collected from 1982 to 2011 or 2014. The diagnoses of PG were based on the characteristic clinical and histological appearances and ruling out of infection. Sixty‐two PG patients, including 29 males and 33 females, were identified. The ages of onset were 16–89 years, and the mean age was 50.2 years. Fifty (80%) of the 62 patients presented with an ulcerative PG, and the lower leg was the most common site (74%). Forty‐six (74%) PG patients had underlying diseases. The most frequent was ulcerative colitis (32%), followed by myelodysplastic syndrome (11%), rheumatoid arthritis (6%) and aortitis syndrome (5%). For treatment, 54 cases (87%) received systemic corticosteroids and 10 received additional treatment with cyclosporin. There was no significant correlation between underlying diseases and response to the initial treatment. Multivariate analysis revealed that the number of affected sites negatively correlated with successful initial treatment. Fifteen (24%) of the 62 cases relapsed. In conclusion, ulcerative colitis and hematological disorders were frequently associated with PG while approximately a quarter of the cases were idiopathic.     

Predicting factors of malignancy in dermatomyositis and polymyositis: a case-control study

BACKGROUND: An association between dermatomyositis (DM)/polymyositis (PM) and malignancies has been widely reported in the literature. The validity of extensive evaluation for malignancies in those patients has also been questioned for decades. Only limited papers regarding the signs of malignancy and the prognostic factors in DM/PM have been reported. OBJECTIVES: To define the potential risk factors of concomitant neoplastic diseases in patients diagnosed as having DM/PM. METHODS: From 1 April 1983 to 30 June 1999, 147 patients were diagnosed as having probable or definite DM/PM at the Veterans General Hospital, Taichung, Taiwan. We excluded four patients who had preceding neoplastic diseases diagnosed before DM/PM, then retrospectively reviewed the data of the remaining 143 patients and subgrouped the cases as four main types: primary idiopathic DM, primary idiopathic PM, juvenile DM/PM and amyopathic DM (ADM). We next performed univariate analysis using logistic regression to evaluate the possible predictive factors for malignancies, such as mean age at onset, gender, manifestations at onset, association with other connective tissue diseases, initial skin presentations, complications and laboratory data. Then we chose the significant factors for multivariate analysis by logistic regression, to determine the independent risk factors of malignancies in DM/PM patients. RESULTS: Among the 143 patients, DM was the most common type (64%), followed by ADM (14%), juvenile DM/PM (13%) and PM (10%). The mean age at onset overall was 42.4 years. Other connective tissue diseases were present in 22% of all patients, especially PM (50%) and juvenile DM/PM patients (28%). Internal malignancies were present in 13% of patients, and most were associated with DM. Nasopharyngeal carcinomas (NPCs) were the most common tumours. Patients with primary idiopathic DM, with an older age at onset, higher serum creatine phosphokinase levels and male gender, had more chance of developing concomitant malignancies. Those associated with complications, especially interstitial lung diseases, had a lower risk of associated neoplasia. In multivariate analysis, an older age at onset (odds ratio 9.10) and male gender (odds ratio 4.06) were associated with greater risk of developing malignancies. CONCLUSIONS: The two independent predictive factors for malignancy (P < 0.05) in patients with DM/PM were an older age at onset (> 45 years) and male gender. The primary idiopathic DM group was shown to have higher risk of developing internal malignancies, especially NPC. However, this was not identified as an independent predictive factor for concomitant neoplastic diseases in multivariate analysis. In addition, patients who had the complication of interstitial lung disease had a significantly lower frequency of malignancies (P < 0.001).     

Sweet syndrome (acute febrile neutrophilic dermatosis) associated with pulmonary coccidioidomycosis

BACKGROUND: Sweet syndrome (acute febrile neutrophilic dermatosis) may arise in association with a variety of underlying systemic diseases. Only 1 case of coccidioidomycosis-associated Sweet syndrome has previously been reported. OBSERVATIONS: We describe 2 patients who developed Sweet syndrome during the onset of acute pulmonary coccidioidomycosis. Systemic antifungal therapy was given in both cases. Respiratory symptoms and skin lesions resolved within 5 weeks. CONCLUSIONS: Sweet syndrome may be a presenting feature of coccidioidomycosis. Recognition of the underlying pulmonary infection is important so that inappropriate treatment with systemic corticosteroids can be avoided.     

Pyoderma gangrenosum: an Indian perspective

Background. Pyoderma gangrenosum (PG) is an uncommon, idiopathic, neutrophilic skin disease sometimes associated with systemic diseases. To our knowledge, there have been no case series of PG reported from India. Aim. To study the clinical features, associated systemic diseases and treatment methods in our case series. Methods. In total, 18 cases of PG diagnosed at our institution in Mangalore were evaluated in a prospective study. Results. Ulcerative PG was the most common variant, seen in all except one case. Paediatric patients constituted less than a quarter (27.77%) of the patients. Systemic diseases were associated with the disease in over half of the patients (55.55%). The pathergy test was positive in seven patients (38.88%), and all but one had associated systemic disease. Corticosteroids were mainly used for management. Conclusions. PG is a rare disease in India, but paediatric PG cases were relatively common. Ulcerative PG was the commonest type, and over half of the patients had associated systemic diseases. There were a significant number of patients with associated systemic disease who had a positive pathergy test.     

Neonatal Autoimmune Blistering Disease: A Systematic Review

We aimed to better understand the pathogenesis, clinical features, prognosis, and treatment of neonatal autoimmune blistering diseases (AIBDs). We searched Medline, Embase, PubMed, Latin American and Caribbean Health Sciences Literature, and reference lists of identified articles. Inclusion criteria were articles published from 1946 to December 2014 in any language. Exclusion criteria were age greater than 4 weeks and no confirmed AIBD diagnosis. We identified 51 cases of neonatal AIBDs: 34 cases of pemphigus (31 pemphigus vulgaris [PV], 3 pemphigus foliaceus [PF]) and 17 cases of pemphigoid diseases (9 bullous pemphigoid [BP], 5 linear immunoglobulin A bullous dermatosis [LABD], 1 BP and LABD, 1 epidermolysis bullosa acquisita, 1 bullous systemic lupus erythematosus). Pemphigoid diseases had a higher male predominance (male:female ratio 4.6:1) than pemphigus (male:female ratio 1:1.06) (p = 0.004). Pemphigus had a higher proportion presenting at birth (79.4%) than pemphigoid diseases (29.4%) (p = 0.008). The most common sites involved were the trunk (63.0%), followed by the head and neck (60.9%). The mucosal membranes were involved in 32.6% of cases (27.6% in pemphigus, 41.6% in pemphigoid diseases). Only 33.3% used systemic therapy, and 75.5% achieved control within 3 weeks. Most PV, PF, and BP cases, but not LABDs, reported maternal disease. In pemphigus cases, 75.0% of mothers had active disease and 25.0% were in control. Pregnant women with PV, PF, and PG of any severity can passively transfer autoantibodies leading to neonatal AIBD. Pemphigoid diseases are more likely to present after birth and may be more male predominant. The presentation of LABDs may be different from that of all other AIBDs.     

培养的自体黑素细胞移植治疗伴自身免疫性甲状腺疾病的非节段型白癜风患者的疗效及安全性随访

【摘要】 目的 探讨培养的自体黑素细胞移植治疗伴自身免疫性甲状腺疾病的白癜风患者的临床疗效及安全性。方法 回顾2008年5月至2018年12月杭州市第三人民医院行培养的自体黑素细胞移植治疗的2 284例非节段型白癜风,其中伴自身免疫性甲状腺疾病75例,包括甲状腺功能亢进42例,甲状腺功能减退18例,桥本甲状腺炎15例。比较伴自身免疫性甲状腺疾病组与不伴自身免疫性甲状腺疾病组的疗效及安全性。计数资料的比较采用χ2检验。结果 2 284例患者中,男1 085例,女1 199例,年龄（25.0 ± 1.2）岁,病程（5.1 ± 2.3）年。术后6个月,2 209例不伴自身免疫性甲状腺疾病组中1 873例有效（84.8%）、1 162例痊愈（52.6%）;伴自身免疫性甲状腺疾病组46例有效（61.3%）、20例痊愈（26.7%）,伴自身免疫性甲状腺疾病组有效率及痊愈率均低于不伴自身免疫性甲状腺疾病组（χ2值分别为29.72、19.54,均P < 0.001）。甲状腺功能减退组有效率低于甲状腺功能亢进组（χ2 = 6.61,P = 0.010）。伴自身免疫性甲状腺疾病组供皮区同形反应发生率（9.3%）高于不伴自身免疫性甲状腺疾病组（4.3%,χ2 = 4.31,P = 0.038）,且移植部位1、3、5及10年白斑复发率（6.7%、14.7%、17.3%、8.7%）均高于不伴自身免疫性甲状腺疾病组（0.7%、1.4%、2.1%、3.6%,χ2值分别为29.96、70.69、67.23、41.61;均P < 0.001）。结论 伴发自身免疫性甲状腺疾病对于白癜风的自体黑素细胞移植治疗具有负相关效应,针对该类患者更应积极采取有效的预防同形反应和移植区复发的手段。     

Acute Hemorrhagic Edema of Infancy: A Troubling Cutaneous Presentation with a Self‐Limiting Course

Acute hemorrhagic edema of infancy (AHEI) is an unusual form of leukocytoclastic vasculitis with dramatic distinguishing skin lesions that occurs in infants ages 4 to 24 months old. The disease presents with skin eruptions that usually start with large (1–5 cm), symmetrically distributed, hemorrhagic lesions in a characteristic cockade pattern. The lesions are typically located on the lower extremities, face (in particular the ears, cheeks, and eyelids), and gluteal area. Fever may accompany skin eruptions. Clinical presentation at onset requires clinical and laboratory examination to distinguish it from more serious diseases and other vasculitis. The main differential diagnosis of AHEI is Henoch‐Schönlein purpura. AHEI is generally a self‐limiting disease, so a conservative approach should be considered. Topical or systemic corticosteroid therapy has been reported to be beneficial, as well as antihistamines and dapsone, although AHEI usually resolves completely with or without treatment. We report two cases of AHEI and an update of the literature.     

Pyoderma gangrenosum: Clinical presentation and outcome in 18 cases and review of the literature

Background: Pyoderma gangrenosum (PG) is an idiopathic ulcerative neutrophilic inflammatory skin disease characterized by variable clinical presentation and outcome. Because its incidence is low, no prospective randomized controlled trials and only a few large case studies on PG have been reported. Observations: We demonstrate the clinical presentation and outcome in 18 cases with severe and chronic PG. In our 18 patients, the female/male ratio was 3.5:1, the mean onset age was 53.1 yrs (range 23–78); six cases (33%) had associated diseases (inflammatory bowel disease [n = 2, 11%], monoclonal gammopathy [n = 2, 11%], rheumatoid arthritis [n = 1, 6%], diabetes mellitus [n = 1, 6%]). Anatomic locations involved were lower leg (n = 14, 78%), abdomen (n = 5, 28%), arm (n = 3, 17%), breast (n = 2, 11%), and buttocks (n = 1,6%). Five patients (28%) had multiple lesions (n ≥ 2). Immunosuppressive monotherapies (n = 3, 17%) and polytherapies (n = 15, 83%) were used. 13 patients (72%) showed complete remission (mean duration to complete remission: 1.29 yrs), three patients (17%) persistent disease (mean duration: 8 yrs), contact was lost to one patient (6%) and one patient died (6%). Conclusion: Our observations add to the growing body of evidence that PG responds in most cases to systemic immunosuppressive treatment, with corticosteroids and cyclosporine representing first‐line therapies. Besides reporting the clinical outcome in our 18 patients, we review the literature and discuss treatment recommendations that take additional factors including associated conditions, disease severity and localization of lesions into consideration.     

Eruptive Melanocytic Nevi: A Review

Eruptive melanocytic nevi (EMN) is a phenomenon characterized by the sudden onset of nevi. Our objective was to compile all published reports of EMN to identify possible precipitating factors and to evaluate the clinical appearance and course. We conducted a systematic bibliographic search and selected 93 articles, representing 179 patients with EMN. The suspected causes were skin and other diseases (50%); immunosuppressive agents, chemotherapy or melanotan (41%); and miscellaneous, including idiopathic (9%). The clinical manifestations could largely be divided into two categories: EMN associated with skin diseases were frequently few in number (fewer than ten nevi), large, and localized to the site of previous skin disease, whereas those due to other causes presented most often with multiple small widespread nevi. In general, EMN seem to persist unchanged after their appearance, but development over several years or fading has also been reported. Overall, 16% of the cases had at least one histologically confirmed dysplastic nevus. Five cases of associated melanoma were reported. We conclude that the clinical appearance of EMN may differ according to the suggested triggering factor. Based on the clinical distinction, we propose a new subclassification of EMN: (1) widespread eruptive nevi (WEN), with numerous small nevi, triggered by, for example, drugs and internal diseases, and (2) Köbner-like eruptive nevi, often with big and few nevi, associated with skin diseases and most often localized at the site of previous skin disease/trauma. The nature of the data precluded assessment of risk of malignant transformation.