Esclerosis sistémica sin esclerodermia en pacientes mexicanos. Serie de casos

Systemic sclerosis sine scleroderma (ssSSc) is a form of systemic sclerosis that is characterized by Raynaud's phenomenon (RP), visceral involvement without thickening of skin and anticentromere antibodies (ACA). We studied 10 ssSsc patients with a prevalence of 2%. The clinical signs were: RP 9/10, esophageal manifestations 8/10, pulmonary arterial hypertension 4/10, interstitial lung disease 4/10, cardiac signs 3/10 and ACA 8/10.

Atypical hemodynamic manifestations of cardiac tamponade.

Clinical examination and transthoracic echocardiography play a vital role in the management of patients with pericardial effusion and cardiac tamponade physiology. We report patients in advanced phase 3 cardiac tamponade with variant clinical and hemodynamic presentations. These atypical cardiac tamponade cases include: A patient with severe aortic valve regurgitation who lacked pulsus paradoxus; a patient with systemic sclerosis without hypotension; and a patient with pulmonary hypertension lacking right heart collapse on echocardiography. Recognition of these atypical clinical and hemodynamic manifestations of cardiac tamponade will avoid undue delay in the treatment.     

A study of scleroderma in South Australia: prevalence, subset characteristics and nailfold capillaroscopy

Background: Scleroderma is a systemic rheumatic disorder seen in a wide range of clinical specialties. Aims: To establish the prevalence and mortality rates of scleroderma in South Australia (SA), to determine the relative frequency and characteristics of the three principal subsets (diffuse, limited and overlap), and to examine the role of nailfold capillaroscopy in subset identification and implied prognosis. Methods: Outpatient and discharge diagnostic indexes from five major teaching hospitals in SA were reviewed between February 1987 and November 1993. A total of 215 patients with scleroderma were identified. Case notes of 115 of these patients were reviewed in order to validate scleroderma diagnosis, and subset characteristics such as sex, mean age at diagnosis, extent of skin involvement, internal organ involvement and serology were analysed. Fifty-two of these patients were then examined prospectively to confirm positive discharge diagnosis, and nailfold capillaroscopy was performed on these patients. Results: The point prevalence of scleroderma in SA for 1993 was estimated to be 208/10⁶. This figure is a conservative estimate and is higher than most other reported series. The female to male ratio was 4:1. The majority of patients had limited disease with a ratio of 6:1:1.6 limited vs diffuse vs overlap. Systemic involvement excluding the oesophageal component in limited disease was found predominantly in the diffuse group. Autoimmune serology was positive in 90% of patients, with Scl-70 being more common in diffuse scleroderma, anti-centromere antibody (ACA) in the limited form and anti-ribonucleoprotein (RNP) in the overlap form. Nailfold capillaroscopy was useful in predicting disease-subtype as capillary dilatation was observed predominantly in limited disease, and capillary dropout in diffuse disease. Conclusions: Scleroderma is more common in SA than previously recognised. Limited disease is more common than diffuse or overlap disease, carries a better prognosis and is associated with ACA. Nailfold capillaroscopy is a useful tool in disease assessment and may provide useful diagnostic and prognostic information.     

节段性皮肤僵硬综合征一例

患儿,男,8岁。因右侧股部、臀部及腰部皮肤触之僵硬8年就诊。患者皮损呈单侧分布,伴有轻度多毛和色素沉着。病理活检示表皮过度角化,基底层色素增加,真皮中层胶原纤维增粗、致密,呈均质化,无炎症细胞浸润,附属器未见异常。阿新蓝染色呈阳性。综上诊断为节段性皮肤僵硬综合征。     

SKIN MIGRATION FOLLOWING PERIURETHRAL POLYTETRAFLUOROETHYLENE INJECTION FOR URINARY INCONTINENCE

We present a patient who was treated unsuccessfully for urinary incontinence with the injection of particulate polytetrafluoroethylene (Polytef). Following removal of the resultant periurethral mass she developed fever, malaise, polyathropathy, a raised erythrocyte sedimentation rate, and she had a skin rash which contained refractile foreign material surrounded foreign body giant cells.     

Undifferentiated connective tissue disease and its cutaneous manifestations

Undifferentiated connective tissue disease (UCTD, also named UCT syndrome, latent lupus or incomplete lupus) is regarded as an autoimmune disorder in which signs and symptoms are widely variable and evocative for connectivitis but not sufficiently evolved to fulfil any of the accepted classification criteria for the defined connective tissue diseases. In this paper we describe the case of a 47-year-old woman affected by UCTD according to the preliminary classification criteria supplied by Mosca et al. in 1999.     

应用多功能血管病诊断仪观察系统性硬皮病肢体血流变化

采用多功能血管病诊断仪（ＰＶＬ），观察了３４例系统性硬皮病患者的肢体血流图，发现其波幅显著低于健康人。通过用硬皮病基本方加减并配合脉络宁注射液治疗３个月～１年，患者的血流情况得到了显著改善。临床疗效：显效率为７０．５９％，总有效率为１００％。     

Computerized evaluation of skin stiffening in scleroderma

Modern non-invasive biometrological methods have proven to be helpful in monitoring some skin disorders. The present study was designed to evaluate the mechanical properties of skin in systemic scleroderma. The Cutometer SM 474 was used to measure the rheological properties of the skin in 33 patients suffering from scleroderma. The most prominent abnormality consisted in a marked reduction in skin extensibility. Changes occurring in time objectivate the evolution of the disease. Measuring the mechanical properties of skin in patients with scleroderma may be useful in monitoring the disease and its treatment.