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。     

The spectrum of localized amyloidosis: A case series of 20 patients and review of the literature

Localized deposition of amyloid may occur in individual organs, in the absence of systemic involvement. The reason for localized deposition is unknown, but it is hypothesized that deposits result from local synthesis of amyloid protein, rather than the deposition of light chains produced elsewhere. We identified 20 cases of localized amyloidosis at our institution between 1993 and 2003. There were 11 males and nine females in the group. The mean age at the time of diagnosis was 65.5 years. Organs involved included skin, soft tissues, oropharynx, larynx, lung, bladder, colon, conjunctiva, and lymph node. In six of nine patients typed, the amyloid light chain was lambda. In those patients where follow-up was available (mean 7.6 years), none developed systemic disease. Localized amyloidosis occurs in a variety of organ systems. Evolution into systemic amyloidosis was not seen in our series of patients, supporting the hypothesis of local production of amyloid protein in these cases.     

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.     

肿胀麻醉脂肪抽吸术284例

目的探讨应用肿胀麻醉抽吸术治疗局限性肥胖的安全性和手术技巧。方法术前设计选择隐蔽切口,采用肿胀技术,负压为0.06kPa,选择直径2.8-4.0mm的钝头侧孔吸脂针,沿术前设计抽吸范围分层、隧道内、放射状抽吸脂肪。结果本组284例患者,术后157例随访3-20个月,术区平整对称,切口瘢痕隐蔽细小,效果满意。结论该方法麻醉效果彻底且作用时间较长,出血少,组织损伤小。正确的脂肪抽吸方法和精细操作,是手术成功的关键。