青斑样血管病的诊疗进展

青斑样血管病是一种非炎性、真皮内血管阻塞性皮肤病,以双下肢远端反复出现疼痛性溃疡,网状青斑以及瓷白色萎缩性瘢痕为主要临床表现。本病病因及发病机制不明,可能与机体高凝状态,纤维蛋白溶解障碍和/或与免疫系统疾病相关。目前无特效疗法,主要以缓解疼痛、防止皮损进展为目的。     

Annular lesions of cutaneous sarcoidosis with granulomatous vasculitis

Sarcoidosis is known to be involved in diseases with vasculitis as sarcoid vasculitis. However, vasculitis in cutaneous sarcoidal lesions is extremely rare. Here we describe a case of sarcoidosis with multiple annular skin lesions with granulomatous vasculitis. A 62‐year‐old female was diagnosed with sarcoidosis by chest‐abdominal computed tomographic examination and laboratory tests. The skin lesions had appeared on her lower limbs 2 years before. Physical examination showed multiple infiltrated annular eruptions on the lower extremities. A skin biopsy of an area of erythema showed multiple non‐caseating epithelioid cell granulomas in the dermis and subcutaneous fat and granulomatous vasculitis with fibrinoid degeneration in the subcutaneous fat. There are two types of vasculitis in sarcoidosis: leukocytoclastic and granulomatous vasculitis. Ulcers and livedo were more common in granulomatous vasculitis than in leukocytoclastic vasculitis. The present case had unique annular skin lesions of sarcoidosis with granulomatous vasculitis.     

Cerebral Segmental Polyarteritis of Unknown Aetiology in Sheep

Vasculitis affecting the meningeal elastic arteries was identified in six sheep of various breeds during routine diagnostic investigation. The lesions consisted of multifocal intimal proliferation, thickening of the tunica media, multifocal infiltration of the media by inflammatory cells and more extensive perivascular accumulation of lymphocytes and plasma cells within the tunica adventitia. Fibrinoid necrosis affected an intergyral artery in one sheep. Immunohistochemistry failed to demonstrate pestivirus antigen associated with the lesions and no alternative aetiology was defined. Possible causes of meningeal segmental polyarteritis in sheep are discussed.     

视网膜血管炎的研究进展

视网膜血管炎主要累及青壮年视网膜静脉,动脉累及较少,致盲率较高.积极防治此病意义重大.其发生可能与感染、免疫机制、氧化反应、全身性疾病等有关,通过荧光素眼底血管造影、超声、实验室检查可提高诊断正确率.针对不同的病程,采用药物、激光、玻璃体视网膜手术等治疗措施可改善视功能.就此病的定义、病因、分类、基础研究、诊断及治疗等方面的最新进展进行综述.     

Concomitant intramyocardial and epicardial vasculitis in an autopsied heart allograft for cardiac rhabdomyosarcoma

Primary cardiac tumours are rare, with only one quarter of the patients being malignant. The vast majority of malignant neoplasms of the heart are sarcomas. We describe a patient of primary cardiac rhabdomyosarcoma presented as coronary artery disease and recurrent myocardial infarction. Histopathologic finding of the excised native heart revealed a high grade pleomorphic rhabdomyosarcoma in the myoepicardial portion of the anterior wall with rupture. The accompanying unusual feature was myocardial infarction because of tumour emboli of the left anterior descending and left circumflex coronary arteries. After transplantation, the patient developed mild to moderate acute cellular rejection of the transplanted heart on post-transplantation day 1, 8, and 44, respectively. Unfortunately, he expired on the post-transplantation day 47 because of acute rejection, presenting as concomitant intramyocardial and epicardial lymphocytic vasculitis and multifocal myocardial ischaemia. We found that this uncommon medial lymphocytic vasculitis lesion was mediated by T cells and also by antibody directly against smooth muscle cells of small arteries. The consequence of such immune response would be compromised myocardial oxygenation resulting in allograft failure.     

Intractable livedoid vasculopathy successfully treated with hyperbaric oxygen

We describe a new method for treating livedoid vasculopathy. The typical presentation of livedoid vasculopathy includes chronic, recurrent painful ulcers, satellite scar-like atrophy and telangiectasia involving the lower extremities. Histologically, these lesions show areas of ulceration and dermal vessel occlusion without frank inflammatory cell infiltration. There is currently no satisfactory therapy available for this disease. Hyperbaric oxygen (HBO) has recently established itself as one of the most effective methods of treating ischaemic wounds, including diabetic ulcers. We used this therapy in two patients whose lesions were resistant to multiple therapeutic modalities. Not only did their ulcers respond rapidly to the HBO therapy, but the disturbing wound pain also resolved at the same time. To our knowledge, this is the first successful trial of HBO therapy in livedoid vasculopathy. We believe this to be a very promising new therapy for livedoid vasculopathy and to be worth further investigation.     

Aortitis during intraarterial chemotherapy for cervical cancer

A 76-year-old woman with stage IIb cervical cancer with a bulky tumor experienced aortitis during continuous intraarterial cisplatin-based chemotherapy. The chemotherapy was administered through a catheter tip placed in the aorta abdominalis, utilizing an external infusion pump. During the third course of chemotherapy, she complained of left-sided lower back pain and moderate fever was observed. Elevated white blood cell count (WBC) and C-reactive protein (CRP) level were noted, and an abdominal X-ray and urgent computed tomography (CT) were performed. The catheter tip was displaced against the arterial blood flow. At this level of the aortic wall, soft tissue density surrounded the aorta completely. Aortitis caused by the intraarterial chemotherapy, was strongly suspected. It was thought that the maldistribution of drugs and changes in the drug flow occurrred due to the vertebral height movement of the catheter tip against the aortic blood flow, and there, flow to the vasa vasorum may have occurred. Chemical vasculitis of the vasa vasorum due to the anticancer drugs was strongly suspected as a contributing factor of the aortitis. Because of the long-term use of an intraarterial catheter, the maldistribution of drugs and changes in the drug flow occurred physically and biologically during the course of the chemotherapy. We recommend occasional monitoring of the location of the catheter tip and a repeat evaluation with contrast medium in regard to flow to the vasa vasorum. Received: August 29, 2001 / Accepted: December 17, 2001     

Anti-heparan sulfate antibodies in neurological disease

Antibodies to heparan sulfate (HS) have previously been found in association with peripheral neuropathy. We tested sera from patients with neuropathies and with other neurological diseases for antibodies to HS using an avidin-biotin enzyme-linked immunosorbent assay (ELISA) system. Increased titers of anti-HS antibodies were found in 3.4% of patients with neuropathy, and in 3% of patients with other neurological diseases. In all cases, however, an inflammatory disease was present, including chronic inflammatory neuropathy, cerebral vasculitis, or multiple sclerosis. Antibodies to HS appear not to be specific for neuropathy, as they occur in several inflammatory diseases. They might contribute to the associated breakdown of the blood-brain or blood-nerve barrier.     

Wegener granulomatosis – an atypical case

 An unusual sequence of the clinical manifestations of microvascular disease is described in a 15 year-old girl. She initially presented with acute renal failure caused by a crescentic glomerulonephritis associated with positive tests for MPO-ANCA. Eighteen months later she had pulmonary hemorrhage and respiratory failure. An open lung biopsy showed granulomas that were diagnostic for Wegener granulomatosis. We discuss the diagnostic dilemmas faced in attempts to distinguish infective causes of pulmonary granulomas, such as tuberculosis or fungi, from granulomas associated with vasculitis, in a patient previously treated with immunosuppressive therapy. Received: 4 August 1999 / Revised: 28 October 1999 / Accepted: 3 January 2000