结节性多动脉炎的皮肤病理分析

本文报道了10例皮肤型结节性多动脉炎,所有病例经过皮肤活体检查,证实均具有结节性多动脉炎的典型图象,本文作了简要描写和分析。疼痛性皮下结节是本组病例唯一的症状,皮损绝大部分发生于四肢(89％)本病系统症状轻微,病程良性,预后良好。     

血管炎的实验室检查

血管炎的实验室检查，不仅对肯定血管炎诊断有一定的价值，而且在分析病因和发病机制，指导临床分类诊断以及治疗均有较重要的意义。1常规检查包括血尿常规、血沉、肝肾功能等。1．1血常规白细胞总数升高和中性粒细胞比例增加，不仅见于细菌感染引起的血管炎，更多可见于急性发热性嗜中性皮病、结节性多动脉炎、Wegener肉芽肿、白塞病、荨麻疹性血管炎等。嗜酸粒细胞升高见于嗜酸性蜂窝织炎、面部肉芽肿、Wegener肉芽肿、变应性皮肤血管炎、结节性多动脉炎。贫血见于严重的变应性皮肤血管炎、结节性多动脉炎、Wegener肉芽肿、白塞病等。在各种类型的血管炎中血小板的质和量均基本正常或暂时性降低。     

皮肤型结节性多动脉炎1例

结节性多动脉炎系一少见全身性疾病。其主要病变为多个器官或系统的坏死性中小动脉炎。本病以系统型多见,皮肤型少见[1],我科收治1例,报告如下。患者男,24岁,工人。因躯干、四肢皮肤起痛性结节,伴发热、乏力1月于1995年6月7日入院。患者2年前皮肤起痛性结节伴发热,诊断为结节性血管炎,先后用氨苯砜及抗痨治疗无效。后经北京某医院行组织活检,诊断为皮肤型结节性多动脉炎,并予强的松辅以中药治疗1月痊愈。本次入院体检:Ｔ38.5℃,系统检查未见异常。皮肤科情况:躯干、四肢可触及散在或簇集分布的黄豆大小暗红色皮下结节,结节大多分布于血管近…     

皮肤型结节性多动脉炎1例

皮肤型结节性多动脉炎(cutaneous polyarteritis nodosa,CPAN)临床少见,笔者遇1例经临床病理确诊,现报告如下。1临床资料     

皮肤型结节性多动脉炎1例

结节性多动脉炎是罕见的,以侵犯小、中等肌性动脉为主的节段性坏死性血管炎,根据系统是否受累,可分为良性皮肤型和系统型.现报告1例皮肤型结节性多动脉炎. 1病历摘要 患者男,46岁,农民.因双下肢、臀部多发性溃疡伴疼痛8个月就诊.患者于8个月前无明显诱因双小腿出现数个黄豆大的红色结节,伴有疼痛,后结节破溃形成溃疡,逐渐向深层发展,数量增多,向上累及臀部,皮损疼痛加重,多次就诊于当地医院,诊断为皮肤溃疡,予以抗生素类药物口服和外用,效果欠佳.患者病程中无发热、肌痛、关节痛、神经痛等症状.既往无遗传病史,家族中无类似疾病史.     

伴有血管炎的自身炎症性疾病

【摘要】 自身炎症性疾病是一组以固有免疫失衡、周期性发热以及反复炎症发作为特征的遗传性疾病。自身炎症性疾病中常见的皮疹表现有痤疮、坏疽性脓皮病、丹毒、荨麻疹、鱼鳞病、脓疱病等,而血管炎少见。本文对可伴有血管炎表现的自身炎症性疾病做一简要概述。其中,家族性地中海热主要以过敏性紫癜和结节性多动脉炎两种中等及小血管炎为主,Blau综合征以大血管炎更常见,腺苷脱氨酶2缺陷患者也可伴有中等大小及小动脉受累,临床上需和结节性多动脉炎相鉴别,干扰素刺激基因突变相关的婴幼儿血管病以皮肤小血管炎及血管病为主。     

皮肤型结节性多动脉炎伴神经损伤1例

报告１例皮肤型结节性多动脉炎患者伴外周神经损伤，经外科手术探查证明神经纤维解剖结构完整，而肌电图检查患肢神经功能严重受损，作者结合临床和组织病理分析了本病造成周围神经损害的原因。     

乙型肝炎携带者所发生的特殊性丘疹性皮损

乙型肝炎病毒感染可伴有各种皮肤体征和综合征,其中有荨麻疹、血管性水肿、斑丘疹、猩红热样皮疹、可触及性紫癜、原发性混合性冷球蛋白血症、结节性多动脉炎及苔藓样皮炎等.这类皮损可个别地伴发于特殊感染期:潜伏期、前驱期、黄疸期、恢复期或慢性期.     

皮肤型结节性多动脉炎并发周围神经炎

报告1例皮肤型结节性多动脉炎并发周围神经炎。患者女,40岁。双下肢斑疹、紫癜、结痂伴麻木疼痛5年,加重5个月。皮肤科检查:双小腿、足背散在褐黄色胡椒粉样色素沉着,部分呈环状分布。双踝内、外侧有可触及的紫癜、点状坏死、结痂、象牙白色萎缩性瘢痕。皮损组织病理:真皮浅层小血管栓塞,真皮深层中等大小动脉周围淋巴细胞和组织细胞浸润,伴管腔纤维蛋白样坏死。左侧腓肠神经组织病理:重度轴索性周围神经改变。该患者诊断为皮肤型结节性多动脉炎并发周围神经炎。给予泼尼松和羟氯喹抗炎,西洛他唑和胰激肽原酶肠溶片等抗血小板聚集、扩血管,甲钴胺和呋喃硫胺营养神经治疗后,皮损基本痊愈,神经症状部分缓解。随访1年无复发。     

结节性多动脉炎和癌

Godeau等最近曾报告二例结节性多动脉炎伴有癌症,一例男性伴有支气管未分化癌,另一例女性有直肠腺癌。本文报告一例男性结节性多动脉炎,有继发性多神经炎,表现为肢体感觉异常,尤以下肢为甚,不久出现运动障碍,左足及右手1、2、3指不能运动。神经科检查发现两侧腓总神经(以左侧更为显著)及右侧正中神经有感觉运动机能障碍。胸片正常。活组织检查证实为结节性多动脉炎。给皮质类固醇激素治疗后症状很快好转,但2年后有低热、咳嗽、咯血,支气管镜检查发现左叶支气管有上皮癌。活检可见有未分化上皮细胞索,有时在棘层内有角化病变。病人给予钴治疗,很快因患者头痛不断加重而发现头顶骨有骨瘤转移。     

Myasthenia gravis with cutaneous polyarteritis nodosa

Cutaneous polyarteritis nodosa presents with typical cutaneous lesions of polyarteritis nodosa without visceral involvement at the time of diagnosis. We report a 32-year-old woman with a 12-year history of myasthenia gravis under immunosuppressive treatment, who presented with tender, erythematous nodules 20 mm in diameter on her shins. A diagnosis of cutaneous polyarteritis nodosa was made, based on the clinical and histopathological findings. Myasthenia gravis, an antibody-mediated disease, is occasionally accompanied by other autoimmune diseases, but we found no previous report in the literature associating it with polyarteritis nodosa.     

Relapsing cutaneous polyarteritis nodosa associated with streptococcal infections

Background Polyarteritis nodosa is an aggressive, often fatal form of vasculitis associated with multi-organ involvement. Cutaneous polyarteritis nodosa is purported to be a more benign form of this disorder with involvement limited to the skin. Methods The identification of a female patient from childhood to adulthood documenting repeated episodes of cutaneous polyarteritis nodosa following bouts of recurrent streptococcal pharyngitis. Results Repeated bouts of streptococcal pharyngitis at ages 11, 28, and 33 years were followed by episodes of cutaneous polyarteritis nodosa, documented by histopathologic skin changes and clinical presentation, and confirmed by therapeutic management. Conclusions Various infectious and non-infectious conditions have been linked both to the initiation and relapse of this disease. We describe a patient with recurrent episodes of cutaneous polyarteritis nodosa spanning a period of over 20 years with each episode appearing to be linked to a prior streptococcal infection.     

CUTANEOUS POLYARTERITIS NODOSA

Summary.— The case histories of 102 consecutive patients suffering from polyarteritis nodosa were reviewed. The 17 patients with skin lesions were personally examined and investigated. Thirteen were found to have polyarteritis affecting the skin. In one of these cutaneous involvement occurred only 4 days before death and 5 weeks after the onset of the illness. The other 12 showed remarkably similar clinical and pathological features and, together with 3 cases from another hospital, are described in detail.
Only the skin, skeletal muscles and peripheral nerves were involved. No deaths have occurred to date (longest follow-up period 23 years), and the disease is now either entirely quiescent or represented only by occasional cutaneous lesions.
It is concluded that about 10% of all cases of polyarteritis nodosa, and 30% of those proven histologically, are of this benign type and that this variety can be easily recognized clinically.     

Cutaneous polyarteritis nodosa. Report of a case associated with Crohn's disease

A patient had cutaneous polyarteritis nodosa associated with Crohn's disease. Immunopathologic studies disclosed evidence of circulating immune complexes as measured by C1q binding. Immunofluorescent microscopy of lesional skin showed C3 and fibrin deposition. Histamine injection of normal skin and subsequent biopsy specimens and immunofluorescent microscopy showed immune deposits. The patient was treated with cyclophosphamide and prednisone with a good clinical response, but within two years, histiocytic lymphoma developed. There is an association of cutaneous polyarteritis and Crohn's disease, and also, immunosuppressive therapy plays a role in the development of histiocytic lymphoma.     

Cutaneous polyarteritis nodosa: A report of 16 cases with clinical and histopathological analysis and a review of the published work

Sixteen cases of cutaneous polyarteritis nodosa referred to our Department from 1985 to 2003 were studied clinically and histopathologically. Laboratory data, treatments and clinical courses were also evaluated retrospectively. All cases had nodules and/or indurated erythemas on their lower extremities. All cases showed necrotizing vasculitis of small muscular arteries in the subcutaneous tissues and/or occlusion of those arteries histopathologically. Fifteen cases also had accumulation of plasma protein in vessels of the dermis and subcutaneous tissues. Laboratory data showed high activity of platelets and coagulation in some cases. Eleven cases had been effectively treated with non-steroidal anti-inflammatory drugs. Eight cases were observed for at least 5 years (the longest for ∼19 years) and had good prognoses and no systemic involvement. Cutaneous polyarteritis nodosa seems to be a benign disease, and differs from systemic polyarteritis nodosa although their histopathological features are common. Cutaneous polyarteritis nodosa might involve local dysfunction of the circulation from the dermis to the subcutaneous area. A review of the published work shows that the cause(s) of most cases of cutaneous polyarteritis nodosa is unknown, that no controlled trials for treatment of cutaneous polyarteritis nodosa compared to polyarteritis nodosa have been reported, and that no definitively effective therapy for cutaneous polyarteritis nodosa has been established.     

Three cases of polyarteritis nodosa cutanea and a review of the literature

We describe three cases of polyarteritis nodosa cutanea (PNC) showing necrotizing arteritis and only cutaneous lesions without systemic symptoms or visceral involvement for eleven, six, and three years after the onset of the disease. Since it was first described, there has been continuous controversy as to whether PNC progresses to systemic PN. Some cases have been described which had begun with a cutaneous lesion and progressed to the systemic form 19 and 18 years after the onset of the disease, so we believe that long term follow-up of this disease is essential.     

Crohn's Disease with Cutaneous Polyarteritis Nodosa in a Child: A Case Report

A 10-year-old boy presented with a 1-day history of multiple painful erythematous skin lesions on his upper and lower extremities. He was admitted to the Department of Pediatrics with persistent right lower abdominal pain and diarrhea. Punch biopsy of a skin lesion on his lower leg showed necrotizing granulomatous vasculitis with septal panniculitis consistent with polyarteritis nodosa, and our differential diagnosis included cutaneous manifestations of Croh''s disease. Abdominal ultrasonography revealed distended colonic loops suggestive of inflammatory bowel disease. Upper and lower gastrointestinal endoscopy revealed lesions involving the duodenum, cecum, colon, and rectum. He developed multiple perianal fistulas during hospitalization. Additional laboratory tests revealed positive results for anti-saccharomyces cerevisiae and antinuclear antibodies. Based on his clinical presentation and laboratory findings, he was diagnosed with Crohn''s disease associated with cutaneous polyarteritis nodosa. We report a rare case of a child who presented with cutaneous polyarteritis nodosa as an extraintestinal manifestation of Crohn''s disease.     

The wide clinical spectrum of polyarteritis nodosa with cutaneous involvement

In order to assess the extent of systemic disease which may accompany polyarteritis nodosa when it involves the skin, fourteen cases of polyarteritis nodosa have been reviewed in whom there was histologically proven cutaneous involvement. It is concluded that a sub-group of polyarteritis nodosa (in which the disease remains limited to the skin, muscles and peripheral nerves) can not necessarily be identified; however, predominant cutaneous involvement suggests a good prognosis.     

Cutaneous rheumatoid vasculitis.

Rheumatoid arthritis is a multisystem disease, with many clinical forms bearing close resemblance to systemic lupus erythematosus, dermatomyositis or polyarteritis nodosa. Although the involvement of the integument is not as disabiling as the joint disease, the extent of disability may be of sufficient magnitude to necessitate therapeutic intervention. Two patients are presented whose skin involvement was suggestive of cutaneous angiitis and who responded dramatically to treatment using sulfasalazine.     

Microscopic polyarteritis. Report of a case with cutaneous involvement and antimyeloperoxidase antibodies.

BACKGROUND--Microscopic polyarteritis is a systemic small-vessel vasculitis that primarily involves the kidneys but may also affect the skin and other organ systems. This unique vasculitis represents one of the vasculitides with antineutrophil cytoplasmic antibodies, usually of the perinuclear immunostaining pattern (P-ANCA, antimyeloperoxidase antibodies). The objective of this case report is to describe microscopic polyarteritis, the use of antineutrophil cytoplasmic antibodies, and the unique cutaneous histopathologic features of our patient. OBSERVATIONS--We describe a patient with clinical findings consistent with microscopic polyarteritis, the presence of antimyeloperoxidase antibodies, and a specific cutaneous leukocytoclastic vasculitis. This report further characterizes the histopathologic features by demonstrating that the anatomic location of the cutaneous vasculitic lesions is at the level of the dermal arteriolar vessels and postcapillary venules. CONCLUSIONS--Cutaneous arteriolar leukocytoclastic vasculitis may prove to be a histologic hallmark of microscopic polyarteritis when it presents in the skin. However, further case comparisons and histopathologic investigations are needed. The consequences of this systemic vasculitis, if not adequately treated, may be life threatening. Recognition of clinical features, use of antineutrophil cytoplasmic antibodies, and demonstration of a cutaneous arteriolar leukocytoclastic vasculitis may aid in the diagnosis and subsequent treatment of patients followed up by dermatologists for vasculitic ulcers.