Collagen V nasal tolerance in experimental model of systemic sclerosis

Our aim was to study skin remodeling and autoantibody production in an experimental model of scleroderma (SSc), following nasal tolerance with human type V collagen (Col V). Female New Zealand rabbits (n = 12) were immunized with two doses of 1 mg/ml of Col V in complete Freund's adjuvant and additional two boosters in incomplete Freund's adjuvant to induce SSc. After 150 days, half of these immunized rabbits were submitted to type V collagen-induced tolerance receiving a daily nasal administration of 25 mug of Col V. Control animals (n = 6) were only submitted to type V collagen-induced tolerance. Serial skin biopsies were performed on days 0, 150 and 210, and stained with H&E, Masson's trichrome and Picrosirius for morphological and morphometric analysis. Types I, III and V collagen were identified by immunofluorescence. The animals' serum samples were collected to determine anti types I, III, IV and V collagen and antinuclear antibodies (ANA). Skin biopsies from immunized animals confirmed SSc morphology as previously described, such as progressive decrease of papillary dermis, appendages atrophy, increased type I, III and V collagen deposition. Rabbits with Col V-induced nasal tolerance showed reduction of skin involvement, with significant decrease of collagen amount. Humoral immune response did not change with nasal tolerance. Collagen V nasal tolerance promotes regression of skin remodeling process in an experimental model of SSc. We suggest that nasal tolerance with type V collagen can be a promising therapeutic option to treat scleroderma patients.     

硬皮病患者皮损转化生长因子β1、β2 mRNA的表达

目的 探讨转化生长因子(TGF)β1和β2在硬皮病发病中的作用。方法 采用原位逆转录聚合酶链反应技术检测17例硬皮病患者皮损中TGF-β1、β2mRNA的原位表达情况,并以10例正常人皮肤组织作为对照。结果 TGF-β1mRNA在硬皮病组表达阳性率和表达程度均高于对照组(P<0.05);TGF-β2mRNA在硬皮病组表达阳性率和表达程度与对照组相近(P>0.05);硬皮病皮损中TGF-β1mRNA表达阳性率和表达程度均高于TGF-β2mRNA(P<0.05).结论 TGF-β1mRNA在硬皮病皮损中高度表达,提示其在硬皮病病理纤维化的形成过程中起一定作用。     

Características clínicas y epidemiológicas de los pacientes con esclerodermia

IntroductionScleroderma is a term used to refer to a group of fibrosing disorders affecting the skin. Two types of scleroderma can be differentiated: systemic sclerosis (SS), with visceral involvement, and localised scleroderma or morphea, confined to the skin and underlying tissues. Few studies have evaluated the characteristics of these patients. The aim of this study was to analyse demographic and clinical features of patients with a diagnosis of scleroderma.Material and methodsAn analysis was performed on the data from adult patients with a diagnosis of scleroderma and followed-up in a single speciality clinic in a tertiary hospital.ResultsThe study included 100 patients (80 with SS and 20 with morphea). The median age of onset was 45 years, and women were more frequently affected than men. Patients were classified according to the subtype of scleroderma. Prevalence of concomitant autoimmune diseases (28% patients) and lichen sclerosus (5%) were also analysed. Another classification is proposed by us for patients with morphea according to the distribution of lesions: isomorphic morphea (lesions on areas of skin friction) and non-isomorphic morphea (other locations). Patients with isomorphic morphea were more predominantly female, with a higher prevalence of overweight and obesity, and a higher association with lichen sclerosus. Patients with non-isomorphic morphea required systemic treatment less frequently.ConclusionsThe present study suggests that a classification for patients with morphea according to distribution patterns could differentiate two subtypes of patients that seem to have differences in disease course.     

Lupus eritematoso cutáneo,morfea profunda y psoriasis en una paciente

Psoriasis is a common inflammatory dermatosis that may be associated with a number of diseases. Recent studies provide evidence that there is a greater frequency of autoimmune diseases, but association with autoimmune connective tissue diseases is uncommon. The coexistence of psoriasis and lupus erythematosus is rare. Besides, the occurrence of morphea has rarely been reported in patients with lupus or psoriasis. We report a woman with cutaneous lupus and morphea profunda associated with psoriasis, with an excellent response to methotrexate, and review the literature.     

Skin elasticity in localized scleroderma (morphoea). Introduction of a biaxial in vivo method for measurement of tensile distensibility, hysteresis, and resilient distension of diseased and normal skin

A method for measuring skin elasticity in vivo based on repeated applications of suction to a circular skin area with a diameter of 10 mm and measurement of tensile distensibility (TD), hysteresis (H), and resilient distension (RD) by the principle of electrical capacitance calibrated to elevation of the skin surface in mm is introduced. Measurements in 14 patients of 51 lesions of localized scleroderma (morphoea) and regional controls revealed a characteristic rheological pattern in the sclerotic state representing structural alterations with reduced TD and H; H became temporarily increased during remission. Measurements in control regions showed significant correlations between TD and H (r=0.382), and between H and RD (r=0.420). Regional differences between extremities, abdomen, and back were significant and “inversely” correlated to skin thickness as measured by ultrasound (r=0.451). The method was highly reproducible according to studies on silicone rubber membrane (SD of TD different sites, 0.023 mm; SD of H, 0.010 mm; SD of TD same sites repeated, 0.010 mm). The biological, day-to-day variation in different regions of a healthy individual was considerably higher (SD of TD, 0.048–0.087 mm), in particular with respect to H (SD, 0.122–0.277 mm). TD was very variable. Experiments on abdominal skin showed that previous mechanical stress resulted in a characteristic pattern with an increase in TD and a decrease in H. Acute edema (histamine weal) resulted in a characteristic pattern with increased TD and H. Both experiments illustrated functional aspects of skin rheology overlying the structure and explaining part of the biological variation.     

超微量肝素对重症新生儿硬肿症的治疗研究

该文应用超微量肝素对重症新生儿硬肿症进行治疗研究，资料表明：治疗组临床症状：体温、哭声低下或不哭、尿少或无尿、呼吸困难、肺出血恢复正常时间以及皮肤硬肿、皮肤苍白或青紫、四肢或全身冰冷、心率增快或减慢以及低血压恢复正常时间均比对照组为早（Ｐ＜０．０１）；吮乳困难与肢体活动恢复正常时间、脉细弱恢复正常时间也均比对照组早（Ｐ＜０．０５）。治疗组血生化检测高钠血症、代谢性酸中毒以及高尿素氮质血症恢复正常时间均比对照组为早（Ｐ＜０．００５）；高钾血症与低血糖症恢复正常时间也比对照组为早（Ｐ＜０．０２）。超微量肝素疗法使用简便、经济、不需凝血检测监护、且安全无副作用、临床易于推广，为今后研究重症新生儿硬肿症提供新的治疗措施。     

Barrett's esophagus complicating scleroderma

Two patients with scleroderma whose esophageal involvement was associated with longstanding reflux esophagitis were found to also have Barrett's esophagus. Since Barrett's esophagus is a premalignant condition, these patients with scleroderma should be considered at high risk for the development of adenocarcinoma of the esophagus.     

系统性硬化症致肺功能损害的探讨

目的 研究系统性硬化症导致肺功能损害的变化。方法 采用美国Ｓｅｎｓｏｒｍｅｄｉｃｓ６２００型人体体积描记仪检测３０例系统性硬化症患者的肺功能。结果 ２８例系统性经症患者有肺功能改变。主要表现为小气道功能改变,气道阻力增高,且两者存在负相关性（Ｐ〈０．０５）。结论 系统性硬化症引起肺功能改变以小气道功能改变及气道阻力增高为主。