Ghosh Sudip Kumar, Bandyopadhyay Debabrata, Saha Indranil, Barua Jayanta Kumar
Department of Dermatology, Venereology, and Leprosy, R.G. Kar Medical College, 1, Khudiram Bose Sarani, Kolkata, India.
Indian J Dermatol. 2012 May;57(3):201-5. doi: 10.4103/0019-5154.96193.
Systemic sclerosis (SSc) is a multisystem connective tissue disorder of uncertain etiology. The clinical picture is frequently dominated by prominent cutaneous manifestations that have diagnostic and prognostic significance. The objective of the present study was to find out the demographic profile and the relative frequencies and characteristics of different mucocutaneous features of SSc in a group of patients from eastern India. In addition, we sought to compare the frequency and pattern of the findings in the limited versus the diffuse variety of the disease.
This was a cross-sectional, clinical observational study. Consecutive patients of SSc attending the dermatology O.P.D. of a tertiary care hospital of eastern India over 3 years were enrolled to the present study.
A total of 46 patients (41 females and 5 males; mean age 29.6±12.3 years) of SSc were evaluated. Among mucocutaneous manifestations Raynaud's phenomenon was present in 39 (84.8%) patients. Other cutaneous features included dyspigmentation (40, 86.9%), sclerodactyly (38, 82.6%), inability to open the mouth (38,82.6%), mat-like telangiectasia (11,23.1%), fingertip ulceration and scarring (29,63%), cutaneous calcinosis (1,2.2%), digital gangrene in (2,4.3%), generalized pruritus (4,8.7%), cutaneous small vessel vasculitis (2,4.3%), chronic urticaria (2,4.3%), flexion contractures of the fingers (13,28.3%), and amputation of the digits (3,6.5%). Mucosal changes were observed in 10 (21.7%) patients and nail changes were seen in 13 (28.2%) patients. Diffuse cutaneous SSc was noted in 27 (58.7%) patients and limited cutaneous SSc was seen in the remainder. Thirty-six (78.2%) patients tested positive for ANA.
The present study provides a snapshot of the spectrum of the demographic and mucocutaneous manifestations of SSc in the eastern Indian population. We have not observed any statistically significant differences between dcSSc and lcSSc in terms of mucocutaneous manifestations in the studied population.
系统性硬化症（SSc）是一种病因不明的多系统结缔组织疾病。临床症状常以显著的皮肤表现为主，这些表现具有诊断和预后意义。本研究的目的是了解印度东部一组患者中SSc的人口统计学特征以及不同皮肤黏膜特征的相对频率和特点。此外，我们试图比较局限性和弥漫性SSc患者的检查结果频率和模式。
这是一项横断面临床观察研究。连续3年在印度东部一家三级医院皮肤科门诊就诊的SSc患者被纳入本研究。
共评估了46例SSc患者（41例女性和5例男性；平均年龄29.6±12.3岁）。在皮肤黏膜表现中，39例（84.8%）患者出现雷诺现象。其他皮肤特征包括色素沉着异常（40例，86.9%）、指硬皮病（38例，82.6%）、张口受限（38例，82.6%）、席纹状毛细血管扩张（11例，23.1%）、指尖溃疡和瘢痕形成（29例，63%）、皮肤钙质沉着（1例，2.2%）、手指坏疽（2例，4.3%）、全身性瘙痒（4例，8.7%）、皮肤小血管血管炎（2例，4.3%）、慢性荨麻疹（2例，4.3%）、手指屈曲挛缩（13例，28.3%）和手指截肢（3例，6.5%）。10例（21.7%）患者观察到黏膜改变，13例（28.2%）患者出现指甲改变。27例（58.7%）患者为弥漫性皮肤型SSc，其余为局限性皮肤型SSc。36例（78.2%）患者抗核抗体检测呈阳性。
本研究提供了印度东部人群中SSc的人口统计学和皮肤黏膜表现谱情况。在研究人群中，我们未观察到弥漫性皮肤型SSc和局限性皮肤型SSc在皮肤黏膜表现方面有任何统计学上的显著差异。
