15例坏疽性脓皮病临床分析

目的为进一步了解坏疽性脓皮病的临床资料、组织病理特点及治疗方法。方法对15例坏疽性脓皮病(PG)患者进行回顾性分析。结果 15例确诊患者中男9例,女6例,男∶女为1.5∶1;溃疡型PG有10例(66.67%),并发的相关内科疾病有类风湿关节炎、溃疡性结肠炎、慢性乙型肝炎、肺部感染、重度贫血。对溃疡周围卫星灶非溃疡皮损行组织病理检查,发现明显的血管炎性组织病理改变。结论溃疡型PG为其主要临床类型,不同部位取材其组织病理表现不同,沙利度胺联合环磷酰胺治疗PG有效,且随访复发率较低。     

天疱疮51例临床病理分析

目的:分析天疱疮的临床和病理特性。方法:分析51例天疱疮患者的临床病理资料。结果:约64.71%(33/51)患者合并系统性疾病,其中以糖尿病居多,占29.41%(15/51);皮损组织病理和免疫病理检查具有特异性。结论:目前治疗天疱疮最有效的药物为糖皮质激素,联合应用免疫抑制剂可尽快控制症状、减少糖皮质激素用量及其副作用。     

重症药疹58例临床分析

目的探讨重症药疹的致敏药物、发生规律、临床特征和治疗措施。方法对1990年12月-2013年4月本院收治的58例重症药疹患者的临床资料进行回顾性分析。结果 58例重症药疹患者中,以重症多形红斑(SJS,25例,43.10%))最多见。致敏药物以抗癫痫药(25.86%)和抗菌药物(25.86%)为主,其次是解热镇痛药(17.24%)。黏膜损害和肝功能损害是最常见的并发症。重症药疹均给予系统用糖皮质激素治疗,SJS组8例、TEN组4例以及DIHS组2例给予激素联合人免疫球蛋白治疗。结论重症药疹中以SJS居多。抗癫痫药物、抗菌药物以及解热镇痛药是引起重症药疹最常见的药物,系统应用糖皮质激素尤其是联合人免疫球蛋白治疗重症药疹有效。     

白色萎缩10例临床分析

目的:提高对白色萎缩的认识,探讨其诊断依据和有效的治疗方法。方法:对2000年以来我院住院的10例白色萎缩患者的临床资料进行回顾性分析。结果:本病发病以中年以上女性居多,皮损好发于下肢;6例患者血管腔内有透明血栓形成(占60%);达那唑、小剂量阿司匹林、双嘧达莫片治疗效果较好。结论:临床特点及组织病理学检查为诊断本病的依据。     

白色萎缩10例临床分析

目的:提高对白色萎缩的认识,探讨其诊断依据和有效的治疗方法。方法:对2000年以来我院住院的10例白色萎缩患者的临床资料进行回顾性分析。结果:本病发病以中年以上女性居多,皮损好发于下肢;6例患者血管腔内有透明血栓形成(占60%);达那唑、小剂量阿司匹林、双嘧达莫片治疗效果较好。结论:临床特点及组织病理学检查为诊断本病的依据。     

52例重症多形红斑和14例中毒性表皮坏死松解症回顾性分析

目的:分析重症多形红斑(SJS)和中毒性表皮坏死松解症(TEN)的致病因素、临床特征、实验室检查指标以及治疗方法的差异。方法:对2013年1月-2018年1月本院收治的52例SJS和14例TEN患者的临床资料进行回顾性分析。结果:引起SJS和TEN的可疑致敏药物以抗生素为主。发热、肝功能异常和肾功能异常是SJS和TEN最常见的并发症。SJS和TEN患者共有65例系统使用糖皮质激素治疗,35例使用人免疫球蛋白(IVIG)、24例使用环孢素治疗。SCORTEN评分超过3分的有11例。共4例死亡。结论:抗生素是SJS和TEN最常见的致敏药物,肝肾损伤及发热是最常见的临床并发症。系统应用糖皮质激素联合环孢素,早期予以IVIG可能是治疗SJS和TEN的有效手段。     

红皮病型、脓疱型、关节病型银屑病并发11例临床分析

目的:分析重症银屑病的临床特征。方法:对2011-2013年11例红皮病型银屑病、脓疱型银屑病及关节病型银屑病同时并发的患者进行回顾性分析。结果:重症银屑病经治疗,大部分患者皮损缓解较快,而关节炎缓解较慢。结论:关节病型银屑病是治疗难点,以传统抗风湿病药物治疗为主,可同时应用生物制剂以改善病情。     

溃疡性结肠炎并发坏疽性脓皮病八例研究

目的 探讨溃疡性结肠炎(UC)并发坏疽性脓皮病(PG)患者的临床表现、组织病理特点及疗效.方法 2009年7月至2016年7月于北京协和医院住院的8例UC并发PG患者的临床表现、辅助检查及治疗转归等资料.结果 8例中男5例,女3例,男女平均发病年龄分别为30.6岁和35.1岁.8例UC肠道症状发病时间均早于PG的出现时间,平均间隔时间为5年.均为全结肠型,且PG出现时,UC均处于活动期,6例为中度活动,2例为轻度活动.PG均表现为皮肤疼痛性溃疡,7例累及下肢.5例患者的皮损组织病理可见典型血管炎改变.5例患者并发关节痛.8例患者均以糖皮质激素和5氨基水杨酸制剂作为基本治疗,其中3例联合免疫抑制剂或米诺环素,2例接受英夫利西单抗治疗.结论 PG是UC的严重皮肤表现,常出现于全结肠型、疾病活动的UC患者,多累及下肢,皮肤组织病理为典型血管炎改变.     

双针法硬化灌注治疗婴幼儿面部化脓性肉芽肿23例安全性分析

目的寻找一种简单、安全有效的注射方法,治疗婴幼儿面部化脓性肉芽肿。方法采用双针法对化脓性肉芽肿(PG)进行硬化灌注治疗,使PG失血供,达到坏死脱落的目的。观察婴幼儿PG患者的临床治愈率和疗效。结果 23例患儿共治疗24次,均达临床治愈标准,1次治愈率达95.7%,术后无瘢痕或其他明显不良反应。随访最长12个月无复发。结论采用双针法硬化灌注治疗婴幼儿化脓性肉芽肿,安全有效,且操作简单不受剂量限制,是一种安全的、值得推广的治疗方法。     

白癜风243例临床分析

目的:了解白癜风的临床特点、发病诱因及相关实验室检查结果,为探讨其发病机制及制定防治措施提供依据。方法:收集2016年1月-2019年1月我科门诊的243例白癜风患者临床资料,包括性别、年龄、病史、家族史、伴发疾病及实验室检查结果、治疗疗效等,用SPSS 19.0软件进行统计学分析。结果:243例患者中:寻常型227例(93.42%),为发病的主要类型,节段型16例(6.58%);进展期187例(76.95%),稳定期56例(23.05%);患者各部位均可累及但以曝光部位高发;皮损面积轻度最常见(175例,72.02%);48例(19.75%)有阳性家族史,49例(20.16%)有并发疾病,104例(42.80%)有明显诱发因素,其中神经精神因素为主要诱因。60例(24.69%)有微量元素异常,以锌含量降低最常见;28例(11.52%)合并甲状腺功能异常,40例(16.46%)抗甲状腺抗体阳性。结论:精神神经应激、自身免疫异常及家族史可能是白癜风发病的始动因素,病程长、肢端皮损或合并毛发变白及黏膜受累病例的治疗仍面临巨大挑战,系统治疗联合点阵激光局部药物导入及紫外线照射是白癜风有效的治疗方法。     

178例艾滋病皮肤表现的临床分析

目的:探讨艾滋病皮肤粘膜疾病的病种分布、临床表现特点。方法:回顾分析178例艾滋病患者住院期间皮肤粘膜疾病的种类、特征及实验室指标。结果:艾滋病皮肤粘膜损害复杂,以真菌、病毒感染为主,分别为165例(92.7%)、120例(67.4%),疾病种类依次为口腔念珠菌病、口腔毛状粘膜白斑、马内青霉病、带状疱疹等23种。有2种以上皮肤粘膜疾病者128例(71.9%)。91.6%的艾滋病患者皮肤粘膜疾病发生于CD4 T淋巴细胞小于200 cells/μL时,其中发生于小于50 cells/μL占74.7%。结论:艾滋病皮肤粘膜损害以真菌、病毒感染为主,皮损的镜检、真菌培养可作为艾滋病皮损常规检查之一,皮肤马内青霉感染是诊断艾滋病的重要线索,部分皮肤粘膜病变可作为诊断疾病进入艾滋病期的依据之一。     

CEPHALIC PYODERMA GANGRENOSUM (PG)-LIKE LESIONS AS A PRESENTING SIGN OF WEGENER'S GRANULOMATOSIS

Background. Pyoderma gangrenosum (PG)-like lesions can be associated with Wegener's granulomatosis (WG); in rare instances, they could be the presenting sign of WG. The purpose of this study was to elucidate the relationship between these two disorders. Methods. The clinical and histologic data from a case of WG with primary ulcerative lesions of the face were compared with five other cases previously reported in the literature. Results. A predominance among young men was noted. In three patients the histology of the skin ulcerations was suggestive of WG, in the remaining three, including the case described, it was nonspecific. Two cases developed pulmonary WG after the skin lesions cleared, showing that the extent and severity of skin lesions do not necessarily correlate with the systemic disease. Conclusions. When the histology of the skin is nonspecific, the diagnosis of PG or PG-like lesions of the head and neck should not be considered definitive and the patients should be carefully monitored to rule out the possible presence of WG.     

CUTANEOUS MANIFESTATIONS OF SYSTEMIC LUPUS ERYTHEMATOSUS IN A TERTIARY REFERRAL CENTER

Background:

Systemic lupus erythematosus (SLE) is an autoimmune disease with multiorgan involvement. The skin is the second most commonly affected organ. SLE with skin lesions can produce considerable morbidity resulting from painful skin lesions, alopecia, disfigurement, etc. Skin lesions in patients with lupus may be specific (LE specific) or may be non specific (LE non specific). Acute cutaneous LE (Lupus specific) has a strong association with systemic disease and non-specific skin lesions always indicate disease activity for which patients present to rheumatologists and internists. Therefore, a thorough understanding of the cutaneous manifestations of SLE is essential for most efficient management.

Aims:

The aims of this study were to evaluate the patterns and prevalence of skin lesions in patients with SLE and to assess the relationship between skin lesions and other systemic involvement.

Materials and Methods:

At the Department of Rheumatology and Clinical Immunology, IPGME&R in Kolkata, 150 patients with SLE fulfilling the clinical and laboratory criteria of the American Rheumatology Association (updated 1982) were examined and followed-up for cutaneous manifestations between January 2002 and January 2007.

Results:

Skin lesions were important clinical features. About 45 patients (30%) presented with skin lesions although all patients had skin lesions during the follow-up period. Skin changes noted were as follows: Lupus specific lesions: malar rash in 120 patients (80%), photosensitive dermatitis in 75 patients (50%), generalized maculopapular rash in 40 patients (26.67%), discoid rash in 30 patients (20%), subacute cutaneous lupus erythematosus (SCLE) in 5 patients (3.34%), lupus profundus in 5 patients (3.34%). The lupus non-specific lesions were non-scarring alopecia in 130 patients (86.67%), oral ulcers in 85 patients (56.67%), vasculitic lesions in 50 patients (33.34%), bullous lesions in 15 patients (10%), Raynaud''s phenomenon in 10 patients (6.67%), pyoderma gangrenosum in 2 patients (1.34%), erythema multiforme in 10 patients (6.67%), and nail fold infarcts in 2 patients (1.34%); however, mucosal discoid lupus, lichenoid discoid lupus, livedo reticularis, sclerodactyly, etc. were not detected. Patients having lupus-specific skin lesions e.g., malar rash were associated with systemic involvement, whereas those having lupus non-specific skin lesions were associated with disease flare.

Conclusions:

Skin lesions in patients with SLE are important disease manifestations and proper understanding is essential for diagnosis and efficient management.     

坏疽性脓皮病25例回顾性分析

目的　从临床上探讨坏疽性脓皮病 (PG)的合并疾病及预后。方法　回顾性分析了 2 5例坏疽性脓皮病住院患者的临床资料和随访结果。结果　资料显示 60 %以上的患者合并相关疾病。合并血液系统疾病的患者预后差 ,而无合并疾病和合并肠道疾病的患者预后较好。值得注意的是化脓性破坏性病变除了累及皮肤外 ,还可累及内脏系统 ,特别是肺部。结论　临床上建议将PG区分为系统型和特发型两种类型 ,PG是一种慢性疾病 ,病情可有起伏 ,治疗以皮质类固醇激素为主 ,也可以辅助应用免疫抑制剂及抗生素等药物。     

Chronic Pruritus in the Absence of Specific Skin Disease

Chronic pruritus is a major and distressing symptom of many cutaneous and systemic diseases and can significantly impair the patient’s quality of life. Pruritus perception is the final result of a complex network involving dedicated nerve pathways and brain areas, and an increasing number of peripheral and central mediators are thought to be involved. Itch is associated with most cutaneous disorders and, in these circumstances, its management overlaps with that of the skin disease. Itch can also occur without associated skin diseases or primary skin lesions, but only with nonspecific lesions secondary to rubbing or scratching. Chronic itch with no or minimal skin changes can be secondary to important diseases, such as neurologic disorders, chronic renal failure, cholestasis, systemic infections, malignancies, and endocrine disorders, and may also result from exposure to some drugs. The search for the cause of pruritus usually requires a meticulous step-by-step assessment involving careful history taking as well as clinical examination and laboratory investigations. Few evidence-based treatments for pruritus are available. Topical therapy, oral histamine H1 receptor antagonists, and phototherapy with UV radiation can target pruritus elicitation in the skin, whereas antiepileptic drugs, opioid receptor antagonists, and antidepressants can block signal processing in the CNS.     

Pemphigoid gestationis ohne Blasenbildung

BACKGROUND AND OBJECTIVE: Pemphigoid gestationis (PG) is a rare pregnancy-associated autoimmune bullous disease characterized by autoantibodies to the 180 kD bullous pemphigoid antigen (BP180). The clinical spectrum of PG is polymorphic and for diagnostic purposes, a skin biopsy is usually taken demonstrating the deposition of autoantibodies. PATIENTS AND METHODS: From 2 patients, skin biopsies were obtained for histopathologic and immunofluorescence studies. Circulating autoantibodies were characterized by immunoblotting and ELISA using a recombinant form of the immunodominant BP180 NC16 A domain. RESULTS: The 2 PG patients described here did not show blisters but complained about severe itching. In the first case, PG presented in the first trimester of the second pregnancy as an erythema-multiforme-like disease. The second patient developed urticarial plaques a few days after delivery. PG was diagnosed by the detection of autoantibodies against recombinant BP180 NC16 A by immunoblot and ELISA analysis and confirmed by linear deposits of C3 at the cutaneous basement membrane zone on direct immunofluorescence microscopy. Skin lesions healed with oral prednisolone. CONCLUSIONS: In our two patients, non-bullous PG could be diagnosed by serological tests. Immunoblotting and ELISA might be sensitive and specific tools when screening sera of patients with pruritic skin lesions in pregnancy for the presence of autoantibodies to BP180. In some cases, these newer techniques may make a skin biopsy unnecessary.     

Clinicopathologic review of 19 patients with systemic candidiasis with skin lesions

BACKGROUND: A diagnosis of systemic candidiasis is often delayed or missed owing to the absence of sensitive, specific, and timely diagnostic tools. Skin lesions are not common, but they can help to rapidly establish a diagnosis. We report on a 14-year experience of systemic candidiasis with skin lesions in our institution. We report the prevalence, clinical findings, histologic findings, etiologic Candida species, underlying conditions, treatment modalities, and outcomes of the cases and compare them with the previous reports. METHODS: We reviewed the medical records and laboratory data of patients diagnosed with systemic candidiasis from June 1989 to September 2002 at Asan Medical Center, Seoul, Korea. We thoroughly reviewed the data on those patients with characteristic skin lesions. We included the cases in which Candida organisms were either shown or cultured from the skin. We also included the patients who had developed the characteristic rash at the onset of infection if there was no other possible explanation for the rash. RESULTS: Of 53 documented systemic candidiasis cases, 19 (35.8%) had the characteristic skin lesions. Fifteen patients (78.9%) had hematologic problems and were neutropenic. The skin lesions were a maculopapular or nodular rash and plaques. In addition to the trunk and proximal extremities, the rash also involved the face and distal extremities. The rashes were mostly purpuric, not consistently associated with underlying thrombocytopenia but also associated with underlying vascular damage as a result of Candida organisms. The underlying vascular damage also caused intraepidermal necrotic and vesicular change. One case of transepidermal elimination of organisms was newly found. The most common causative species was Candida tropicalis in the 19 patients with skin lesions, in contrast with Candida albicans in a total of 53 patients. The mortality rate was 84.2%. CONCLUSIONS: The prevalence of systemic candidiasis-associated skin lesions may be higher than previously reported. Dermatologists should be familiar with the clinical appearance of skin lesions and suspect this fatal infection when seeing neutropenic patients with a resistant fever and accompanying rash.     

坏疽性脓皮病19例临床分析

目的提高对坏疽性脓皮病(Pyoderma Gangrenosum,PG)的认识,探讨其发病原因、临床特点和治疗方法。方法回顾性分析本科1998年1月~2009年2月收治的19例坏疽性脓皮病的临床资料。结果19例患者中男女比为3.75∶1,11(57.89%)例合并有基础疾病,7(36.84%)例皮损位于小腿,3(15.79%)例皮损泛发,所有病例使用糖皮质激素及局部治疗后7例痊愈,12例好转。结论PG是一种皮肤特发性、溃疡性和炎症性疾病。糖皮质激素和免疫抑制剂为治疗一线用药,局部治疗包括外科换药、抗感染和注射糖皮质激素。     

Drug-induced,Ro/SSA-positive cutaneous lupus erythematosus

OBJECTIVE: To study the clinical and immunopathologic findings of drug-induced, Ro/SSA-positive cutaneous lupus erythematosus (CLE). DESIGN: Retrospective medical and laboratory record review. SETTING: Immunodermatology Division of Johns Hopkins Hospital (Baltimore, Md). PATIENTS: Of 120 patients found to have anti-Ro/SSA antibodies by hemagglutination and/or double immunodiffusion, 70 had clinical and immunopathologic confirmation of CLE. Fifteen of these 70 patients had a history of new drug exposure, defined as less than 6 months, associated with disease development. RESULTS: The disease-associated drugs included hydrochlorothiazide (5 patients), angiotensin-converting enzyme inhibitors (3 patients), calcium channel blockers (3 patients), interferons (2 patients), and statins (2 patients). The most common presentations were photodistributed diffuse erythema and subacute CLE-type lesions without evidence of significant systemic disease. All specimens revealed interface dermatitis and fine granular IgG deposition along the basement membrane zone and throughout the epidermis. Most patients experienced improvement or resolution of clinical lesions within 8 weeks and decrease of Ro/SSA titers within 8 months after discontinuation of drug treatment. CONCLUSIONS: Antihypertensive drugs are the most commonly associated with Ro-positive CLE. Clinical and immunopathologic features of this drug-induced variant do not seem to differ from the idiopathic disease. In most cases, the disease improves or resolves on discontinuation of the offending drug treatment. It is not known if these drugs precipitate disease in patients who have subclinical disease. Drug-induced Ro/SSA-positive CLE should be included on the differential diagnosis in patients presenting with photosensitive or subacute CLE-type eruptions.