Dyphenylhydantoin induced hypersensitivity reaction with an unusual purpuric skin lesion

We describe a boy with an unusual adverse reaction to dyphenylhydantoin (DH). This 12 years old boy developed a clinical picture of high fever, scarlatiniform rash, a hemorrhagic (purpuric) skin lesion, on his buttocks and neck, stomatitis and conjunctivitis, within two weeks after DH administration. Laboratory examinations were compatible with the diagnosis of disseminated intravascular coagulation.Cases similar to ours are reviewed and it is stressed that DH should be used cautiously as it is not a harmless drug.     

First trimester maternal concentrations of thrombin-inhibitor complexes and the presence of histologic placental lesions at delivery

Objective. Placental fetal vessel thrombosis or vasculitis and retroplacental hematoma have been associated with adverse neonatal outcomes. The activation of thrombin may contribute to the development of thrombosis and inflammation, and can be assessed through the measurement of thrombin-inhibitor complexes.

Methods. A nested case–control study was performed within a cohort of women with singleton gestations. Thrombin–antithrombin III (TAT) and Thrombin-Heparin co-factor II (T-HCII) concentrations were measured in first trimester maternal plasma. Cases were defined by retroplacental hematoma and/or fetal vessel thrombosis or vasculitis in the umbilical cord or chorionic plate. Outcomes were analysed with Mann–Whitney U and linear regression.

Results. Concentrations of both TAT (p = 0.013) and T-HCII (p = 0.001) from maternal plasma was significantly lower in cases than in controls.

Conclusions. The development of placental inflammatory and thrombotic lesions at term may be associated with lower concentrations of thrombin-inhibitor complexes earlier in the pregnancy.     

肺原发性小血管炎21例分析

目的探讨肺小血管炎的疾病特点及诊断,为血管炎的临床诊断提供借鉴。方法回顾性分析我院2002年1月至2010年12月21例肺小血管炎患者临床资料,其中12例为ANCA阳性患者,2例为病理确诊患者,余7例为临床诊断患者。结果肺小血管炎的临床表现无特异性;ANCA及病理学检查有助于早期诊断。结论原发性肺小血管炎影像表现无明显特异性,应综合分析提高对该病的认识。     

白塞病的血管损害与免疫学改变

目的：探讨白塞病（ＢＤ）的血管损害以及免疫学改变。方法：对４５例ＢＤ患者的临床及病理学资料进行分析。结果：１４例皮肤组织活检、１例扁桃体活检及１例食道粘膜活检病理改变均符合小血管炎症。２例（２／１２）皮肤直接免疫荧光（ＤＩＦ）阳性，为真皮小血管壁ＩｇＭ和Ｃ３ｃ沉积。４例并发心血管系统损害，其中２例为冠状动脉瘤伴血栓形成。１例为脑栓塞性舞蹈病，１例为食道下段溃疡并出血。有多项免疫学指标异常。１０例检     

Polyneuropathy and systemic vasculitis. An electrophysiological study

A review of case of systemic vasculitis followed for one year in a rheumatology depertment resulted in the detection of 18 patients who on the clinical and electrophysiological evidence had symmetrical distal polyneuropathy. A moderate impairment of the motor conduction velocity was present in 7 patients, with electromyographic neurogenic changes in the distal lower limbs of all but one patient. The sensory action potential of the sural nerve was bilaterally absent in one case, and its amplitude was reduced in 14 out of 16 patients, with a decreased sensory conduction velocity in 9 cases. The sural nerve biopsy, performed in 6 patients, was prevalently suggestive of previous axonal degeneration. This investigation illustrates the spectrum of diffuse peripheral nerve involvement associated with systemic vasculitis. A variable impairment of the sensory action potential, ranging from slight decrease of amplitude to no response, is the most common finding. Conduction velocities appear to be relatively spared.

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Sommario A seguito di una revisione dei casi di vasculite sistemica seguiti nel corso di un anno presso un reparto di Reumatologia, sono stati individuati 18 pazienti con caratteri clinici ed elettrofisiologici di polineuropatia distale simmetrica. Un rallentamento della velocità di conduzione motoria era presente in 7 pazienti, mentre segni EM Grafici di denervazione nei muscoli distali degli arti inferiori erano presenti in tutti i casi, tranne uno. Il potenziale d’azione sensitivo (SAP) del nervo surale era assente bilateralmente in un caso, e di ampiezza ridotta in 14 su 16 pazienti esaminati, con ridotta velocità di conduzione in 9 casi. La biopsia del nervo sulrale, eseguita in 6 pazienti, mostrava prevalenti aspetti di tipo assonopatico. Questo studio documenta gli aspetti di compromissione diffusa del sistema nervoso periferico in corso di vasculite sistemica: dal punto di vista elettrofisiologico, l’alterazione di ampiezza del SAP rappresenta il reperto più comune assieme agli aspetti di denervazione, mentre le velocità di conduzione appaiono relativamente risparmiate.

     

CUTANEOUS VASCULITIS DUE TO CYCLOPHOSPHAMIDE THERAPY FOR CHRONIC LYMPHOCYTIC LEUKEMIA

A 55-year-old male with chronic lymphocytic leukemia developed a skin eruption after receiving oral cyclophosphamide. Subsequent rechallenge with parenteral cyclophosphamide led to recurrence of the skin lesions, which on biopsy were shown to be vasculitic in nature. (Aust NZ J Med 1989; 19: 55–57.)     

原发性小血管炎肺脏受累

目的　探讨原发性小血管炎肺脏受累的诊断和治疗。方法　对 15例原发性小血管炎伴肺脏受累患者的临床资料进行分析。结果　肺脏是原发性小血管炎常受累脏器之一 ,约占原发性小血管炎患者 6 0 % (15 / 2 5例 )。肺脏受累的临床表现有咳嗽、哮喘 ,不同程度咯血 ,胸片呈现多样化。由于肺泡广泛出血导致大咯血窒息及严重低氧血症是肺脏受累的严重并发症 ,若能及时采用血浆置换疗法和 (或 )激素、细胞毒冲击治疗 ,肺出血可迅速吸收。动态观察发现不少病人胸片及临床症状可呈戏剧性变化 ,临床危重症得以改善。结论　原发性小血管炎伴肺脏受累是常见的。适当的治疗可改善其预后。     

Giant Cell Arteritis and Angiogenesis: A Review

Giant cell arteritis is a vasculitis of large and medium sized arteries with variable clinical presentations. Arteritic anterior ischemic optic neuropathy is the most common cause of visual loss from giant cell arteritis and the ischemia related to this process is presumed to be secondary to luminal stenosis from intimal hyperplasia. This process has been found to be initiated and promoted by various inflammatory and pro-angiogenic factors.     

Anticardiolipin antibodies and occlusive vascular disease of the eye: Prospective study

There is a recognized association between the presence of anticardiolipin antibodies and vascular occlusive disease. The purpose of our study is to detect the presence of high titers of anticardiolipin antibodies (ACA) in the serum and to correlate the titers with the severity of the vascular disease in patients with occlusive ocular vascular disease. 82 patients were included in a prospective study; 25 patients with anterior ischaemic optic neuropathy, 36 with retinal vein occlusion and 21 with retinal artery occlusion. ACA (IgG and IgM isotypes) were measured by ELISA in the sera of all patients. The group of the patients (group A) was compared to an age-matched control group of 79 healthy individuals (group B). IgG isotype (but not IgM) titers of ACA were found significantly higher in group A (P < 0; 0.001). In patients with titers of ACA (IgG isotype) > 100 units we noted a higher incidence of a more severe disease (recurrency, involvement of both eyes or extraocular manifestations) especially among those with anterior ischaemic optic neuropathy and secondarily in those with retinal artery occlusion. Our results suggest that the association between high titers of ACA and occlusive vascular disease of the eye concerns only the IgG isotype. In addition, the detection of high titers of IgG/AGA in patients could be a useful marker for disease severity and prognosis and this observation seems to be more explicit in cases with arterial occlusive disease than in cases with venous occlusive disease. This revised version was published online in July 2006 with corrections to the Cover Date.     

Annular vasculitis in association with sarcoidosis.

We report a case of cutaneous vasculitis with annular features in association with sarcoidosis. A 36-year-old woman presented with fever, polyarthralgias, erythema nodosum, bilateral hilar lymphadenopathy, and induration of a long-standing scar on the face. In addition, she developed annular, erythematous, and purpuric patches over her thighs and buttocks that were histologically characterized by a small vessel leukocytoclastic vasculitis. The presence of circulating immune complexes in the early stages of sarcoidosis might be related to the occurrence of the vascular damage.