血管炎——发病机理、病理与免疫病理

血管炎是一种具有血管的炎症和坏死的临床病理过程;在临床上,可仅仅表现为水肿性红斑、斑丘疹、可触性紫癜、触痛性结节、水疱和血疱;亦可伴有或仅有其它系统的症状和体征。在组织病理上,表现为血管内皮细胞肿胀,血管壁及其周围的炎症浸润,纤维蛋白样变性或有肉芽肿性增生(1、2、3)。关于血管炎的历史、命名、分类、临床表现、实验室检查及其治疗等方面的资料很多,本文仅着重对血管炎的发病机理、病理及免疫病理方面做一复习。     

大疱性表皮坏死松解型药疹合并剥脱性皮炎型药疹1例

大疱性表皮坏死松解型药疹合并剥脱性皮炎型药疹１例杨雪燕杨汉法大疱性表皮坏死松解型药疹救治中用丁胺卡那霉素后并发剥脱性皮炎型药疹１例，报告如下。患者女，２１岁，因产后出血性休克、高热于当地卫生院用青霉素、氨苄青霉素、先锋霉素Ｖ、代血浆及其他升压药物（具...     

107例皮肤血管炎临床及组织病理分析

目的总结107例病理确诊皮肤血管炎临床和组织病理特点,指导临床治疗。方法采用回顾性分析的方法对2010至2014年107例皮肤血管炎患者的临床资料及组织病理进行分析总结。结果 1男性37例,女性70例,平均年龄(37.85±18.78)岁。2结节性血管炎46例(42.99%),变应性血管炎22例(20.56%),青斑性血管炎13例(12.15%),过敏性紫癜4例(3.7%),进行性色素性紫癜性皮病9例(8.41%),荨麻疹性血管炎12例(11.21%),脓疱型血管炎1例(0.93%)3所有皮肤血管炎病例发生部位频率最高的为下肢。4临床与病理诊断符合病例数为90例,占所有统计病例的84.11%。结论 5年期间确诊病例数占同期病检总数比率相对稳定,皮肤血管炎临床表现多样,组织病理分型复杂,应注意适当的病检,以指导临床。     

卡马西平所致重症药疹的护理体会

大疱性表皮松解型药疹是药疹中最严重的一型,全身中毒症状严重,伴有高热和内脏病变,如抢救不及时,可死于感染、毒血症、肾衰、肺炎或出血.卡马西平具有抗癫痫作用,由卡马西平引起此型重症药疹病情危重,病死率高,有效的护理可提高临床治愈率,缩短住院时间.我科2008年5月至2010年8月共收治5例服用卡马西平所致重症大疱性表皮松解型药疹的癫痫患者.现将护理体会总结如下.     

皮肤血管炎

血管炎是指组织病理学上血管壁及其周围组织的炎症性改变，包括血管内皮肿胀、红细胞外溢、血管壁及周围有纤维蛋白样物质沉积及炎细胞浸润，严重者有血栓形成、甚至整个血管的破坏。皮肤血管炎可表现出谱系改变，在临床上可表现为红斑、丘疹、紫癜、皮下结节、水疱、血疱、坏死、溃疡等。发生机制包括循环免疫复合物的损害、     

大剂量丙种球蛋白治疗三种重症皮肤病的体会

我们于2003～2005年使用大剂量丙种球蛋白（IVIG）治疗大疱性表皮松解型药疹2例，葡萄球菌性烫伤样皮肤综合征及重症荨麻疹性血管炎各1例，现报道如下。     

大疱性表皮松解型药疹38例临床分析

为分析38例大疱性表皮松解型药疹的临床特征。回顾分析38例大疱性表皮松解型药疹患者的临床资料，重点比较儿童和成人患者的异同。解热镇痛药分别占儿童及成人患者致敏药物的59．1％和31．3％。该型药疹皮损虽重．似心脏损害轻。与成人比较，儿童多始发于面部，较少有口腔黏膜受累、发热及低蛋白血症，对糖皮质激素治疗反应好。解热镇痛药是大疱性表皮松解型药疹最常见的致敏药物。儿童较成人临床表现轻，治疗效果好。     

去痛片致紫癜型药疹1例

患者女,72岁,服用去痛片后双下肢瘀点、瘀斑伴痒7d。皮肤科情况:双下肢见散在大小不等瘀点、瘀斑,呈紫红色,部分瘀斑上可见血疱,无破溃及渗出,压之不退色,无压痛,未见关节肿胀。皮损组织病理示:表皮细胞内水肿,部分角质细胞坏死形成表皮内疱,局灶性基底细胞液化变性,真皮浅层水肿,大量血管外红细胞,真皮血管周围见淋巴细胞及多量嗜酸性粒细胞浸润。结合临床与病理诊断为紫癜型药疹。     

同患两型重症药疹死亡1例

重症药疹临床上少见，而两型重症药疹同发一人临床属罕见。我科收治1例先以剥脱性皮炎型药疹入院后出现大疱性表皮松解型药疹而致病情严重导致死亡的病例。现报道如下。     

药疹患者凝血及抗凝血系统功能状态的研究

为了进一步探讨药疹的发病机理和提供新的治疗途径，应用ＣｏａｔｒｏｎⅣ型血液凝固仪及酶联免疫吸附试验、火箭电泳法测定了７１例药疹患者凝血及抗凝血功能，其中多形红斑型、紫癜型及剥脱性皮炎型ＡＰＴＴ较对照组明显缩短，紫癜型及剥脱性皮炎型ＰＴ明显缩短（Ｐ〈０．０１，Ｐ〈０．０５），ＡＴ－Ⅲ明显减低，Ｄ－ｄｉｍｅｒ显著增高，表明重症药疹时凝血及抗凝血之间的平衡失常。当肝肾功能障碍时，要注意血栓前状态，警惕Ｄ     

Purpura of the ears: a distinctive vasculopathy with circulating autoantibodies complicating long-term treatment with levamisole in children

The cutaneous side-effects of levamisole include non-specific and lichenoid eruptions, fixed drug eruption and, very rarely, cutaneous vasculitis. We describe a distinctive clinical and histological vasculopathy with immunological abnormalities in children with paediatric nephrotic syndrome receiving long-term levamisole treatment. Four boys and one girl were identified. Their average age was 10 years. Levamisole had been used for an average of 24 months. Purpura of the ears was the most common finding corresponding histologically to a vasculopathic reaction pattern ranging from a leucocytoclastic and thrombotic vasculitis to a vascular occlusive disease without true vasculitis but with associated antinuclear, antiphospholipid and anticytoplasmic antibodies. The eruption resolved in all patients 2-3 weeks after the discontinuation of levamisole, but serum autoantibodies persisted for 2-14 months.     

Scabies presenting as a necrotizing vasculitis in the presence of lupus anticoagulant

A 75-year-old man presented with a progressive eruption of 6 weeks' duration. Purpura and focal areas of cutaneous infarction were present on the legs. There were scabies burrows on the wrists and hands, and a mite was extracted. Histology of a skin biopsy showed a necrotizing leucocytoclastic vasculitis. Circulating lupus anticoagulant was detected. Treatment consisted of topical 5% permethrin cream and systemic steroids.     

A four-year-old boy with fever, rash, and arthritis

The triad of fever, rash, and arthritis in a hospitalized child suggests an inflammatory, infectious, or postinfectious process in most cases; however, malignancy must be considered. The most common causes in this age group are inflammatory conditions, including Kawasaki disease, Henoch-Sch?nlein Purpura, serum sickness-like reaction, and juvenile idiopathic arthritis. Other rarer inflammatory processes can present with this triad of symptoms such as Cryopyrin-related diseases (autoinflammatory disorders), urticarial vasculitis, and systemic lupus erythematosus. We will discuss the differential diagnosis and inpatient management of fever, rash, and arthritis in a young child, focusing on inflammatory conditions. The important features which can help distinguish these conditions include the nature of the rash, associated signs or symptoms, time course of the eruption, and characteristic laboratory and/or histologic findings.     

''Dalmatian dog''-like skin eruption (two cases of multifocal fixed drug eruption induced by mefenamic acid)

Mefenamic acid is a common widely prescribed drug with analgesic activity. Authors report two cases of multifocal fixed drug eruption induced by mefenamic acid. Cases were diagnosed on basis of clinical examination and histopathology of skin lesion. Only a few cases have been reported in the literature and these are the first two described in Greece.     

卡马西平致药疹18例临床分析

目的探讨卡马西平药疹的临床特点及治疗。方法对1996年4月~2004年4月本院收治的18例卡马西平药疹的临床资料进行分析。结果18例卡马西平药疹中重症多形红斑型13例(72%),大疱性表皮松解型1例(6%),麻疹样发疹型4例(22%),重症型多有肝、肾功能损害,病程长,皮质类固醇激素治疗有效。结论卡马西平药疹,多为重症药疹,具有多器官受累、临床症状严重、病程长,合理使用皮质类固醇激素是治疗的关键。     

Atenolol-induced cutaneous vasculitis

We present a case of cutaneous vasculitis apparently due to an adverse reaction to atenolol. The causal relationship between the drug and the eruption was based mainly on circumstantial evidence. It has been further strengthened by positive results of the indirect rat mast cell degranulation test. The number of published cases of reaction to atenolol is limited. Cutaneous vasculitis has, to the best of our knowledge, never been reported as an adverse reaction to atenolol, although it is not a rare side effect of other beta blocker drugs including propranolol and practolol. Atenolol should be added to the list of beta blocker medications that may produce cutaneous vasculitis.     

Acute generalized exanthematous pustulosis due to clindamycin

Acute generalized exanthematous pustulosis (AGEP) is a rare skin eruption most commonly caused by medications. It is characterized by fever and the acute eruption of non-follicular pustules overlying erythrodermic skin. Histopathology shows subcorneal pustules with a background of dermal edema and spongiosis, leukocytoclastic vasculitis, perivascular eosinophils, and focal necrosis of keratinocytes. Three cases of clindamycin induced AGEP have been reported in the literature. A case of AGEP due to clindamycin is reported in a patient with numerous other drug allergies and without history of psoriasis. Presentation and treatment of AGEP are reviewed.     

Cutaneous histopathology of Rocky Mountain spotted fever

The dermatologic diagnosis of Rocky Mountain spotted fever (RMSF) is often presumptive; the clinical presentation includes skin rash and febrile illness with or without a clear history of tick bite. The characteristic cutaneous manifestations include a generalized skin eruption with purpuric, blanching or non-blanching macules and papules usually involving the extremities. Although skin biopsies are often performed to confirm the diagnosis, the spectrum of cutaneous histopathology in RMSF has not been well described. We studied a series of 26 cases of RMSF, of which 10 were surgical specimens and 16 were autopsies. The microscopic changes were correlated with the duration of illness. The main histopathologic feature was lymphohistiocytic capillaritis and venulitis with extravasation of erythrocytes, edema, predominantly perivascular and some interstitial infiltrate. Leukocytoclastic vasculitis (LCV) with neutrophilic infiltrate and nuclear dust was seen in 11 of 15 (73%) specimens from involved skin. These lesions with LCV also showed notable epidermal change including basal layer vacuolar degeneration with mild dermoepidermal interface lymphocytic exocytosis. Six lesions with LCV displayed focal fibrin thrombi and capillary wall necrosis. Apoptotic keratinocytes were noted in 3 lesions with LCV. Subepidermal blister was observed in the skin lesion of an autopsied patient with LCV changes. Another lesion of a fetal case with LCV also contained features of acute neutrophilic eccrine hidradenitis. Focal small nerve twig inflammation was noted in a third autopsy case with LCV. Plasma cells were seen in 6 of 34 specimens (18%); and eosinophils were observed in 3 (9%). The subcutaneous fat contained a mild perivascular inflammation and one case revealed focal lobular neutrophilic inflammation. Immunohistologic (IH) staining using polyclonal rabbit anti-Rickettsia rickettsii demonstrated positive staining of the organisms in the affected endothelial cells in all 12 cases tested. The cutaneous histopathology of RMSF is caused by endothelial damage by the rickettsial organisms which elicit an initial lymphohistiocytic small vessel vasculitis with progression to LCV. The vasculitis in RMSF is, therefore, considered to be a form of septic vasculitis.     

Autoimmune progesterone dermatitis manifesting as generalized fixed drug eruption

Autoimmune progesterone dermatitis is an uncommon, poorly recognized and under-diagnosed catamenial dermatosis associated with hypersensitivity reactions to progestagens. Most cases manifest as urticaria, eczema or erythema multiforme-like. A 26-year-old woman developed violaceous plaques on the groin and abdomen, 4 days after a spontaneous abortion resolved with uterine curettage. The lesions recurred once monthly at the same sites, mimicking a fixed drug eruption. Although the histopathology was compatible with fixed drug eruption, positive intradermal testing and symptomatic improvement after using oral contraceptive pills gave us a clue to the diagnosis.     

Ibuprofen-induced bullous leukocytoclastic vasculitis

A dramatic case of ibuprofen-induced bullous leukocytoclastic vasculitis (LCV) is described in a patient with a history of prior sensitization to ibuprofen, a common household nonsteroidal anti-inflammatory drug (NSAID) that has few reported adverse skin reactions. Bullous LCV is a relatively rare clinical presentation of LCV, which requires differentiation from other blistering diseases, including bullous erythema multiforme, bullous fixed drug eruption, linear IgA bullous dermatosis, and bullous pemphigoid. The distinctive histopathologic changes of leukocytoclastic vasculitis readily distinguish this bullous eruption from the others.