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系统性硬化症中的皮肤受累情况。

Skin involvement in systemic sclerosis.

作者信息

Czirják L, Foeldvari I, Müller-Ladner U

机构信息

Department of Immunology and Rheumatology, University of Pécs, Pécs, Hungary.

出版信息

Rheumatology (Oxford). 2008 Oct;47 Suppl 5:v44-5. doi: 10.1093/rheumatology/ken309.

DOI:10.1093/rheumatology/ken309
PMID:18784142
Abstract

Skin thickening is a characteristic feature of SSc. More extensive skin involvement coincides with more severe internal organ manifestation(s), poor prognosis and increased disability, at least in the early phase of the diffuse cutaneous scleroderma subset. The fully validated, feasible method ('gold standard') for measuring the dermal skin thickness is the modified Rodnan skin score (mRSS). The responsiveness of mRSS was somewhat modest in clinical trials, and a careful teaching process is necessary. Parallel method(s) for measuring skin thickness need to be used in the future. Ultrasound (US) measurement of the dermis with a 20-30 MHz probe is a valid, reproducible and responsive method in patients with dcSSc. However, US is time-consuming and requires a training process. Of the mechanical instruments available, only the durometer, which measures the hardness of skin, has been validated. The inter- and intra-observer reproducibility and sensitivity to change of durometry were good, and correlated with mRSS and US-measured skin thickness. Several further mechanical instruments exist including the elastometer, twistometer, cutometer and plicometer. They seem to distinguish between involved and non-involved skin, and therefore merit further evaluation. The measurement of late-stage, irreversible skin damage/atrophy should be resolved in the future through the development and validation of new instruments.

摘要

皮肤增厚是系统性硬化症(SSc)的一个特征性表现。至少在弥漫性皮肤型硬皮病亚型的早期阶段,更广泛的皮肤受累与更严重的内脏器官表现、预后不良及残疾增加相关。用于测量真皮皮肤厚度的经过充分验证且可行的方法(“金标准”)是改良的罗德南皮肤评分(mRSS)。在临床试验中,mRSS的反应性略显一般,因此需要进行仔细的培训。未来需要使用并行的皮肤厚度测量方法。使用20 - 30MHz探头的超声(US)测量真皮厚度,对于弥漫性皮肤型系统性硬化症(dcSSc)患者是一种有效、可重复且有反应性的方法。然而,超声测量耗时且需要培训。在现有的机械仪器中,只有用于测量皮肤硬度的硬度计经过了验证。硬度计在观察者间和观察者内的可重复性以及对变化的敏感性良好,并且与mRSS和超声测量的皮肤厚度相关。还存在其他几种机械仪器,包括弹性计、扭转计、皮肤弹性测定仪和皱纹计。它们似乎能够区分受累皮肤和未受累皮肤,因此值得进一步评估。晚期不可逆皮肤损伤/萎缩的测量问题未来应通过开发和验证新仪器来解决。

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