OBJECTIVE: To report the experience using the Total Skin Score (TSS), in the original (OR-TSS) and modified (MR-TSS) Rodnan methods in systemic sclerosis (SSc), analysing its clinical significance in the model of two (diffuse and limited) and three (diffuse, intermediate and limited) clinical variants. METHODS: Prospective study analysing skin thickness (assessed by original and modified Rodnan TSS methods) in 56 SSc patients. TSS results were compared with SSc clinical variants (subdivided in the model of two and three subgroups) as well as with cutaneous (calcinosis, telangiectasias, pigmentary abnormalities), systemic (articular, vascular, esophageal, pulmonary, cardiac, renal) and laboratory (antinuclear antibody, anticentromere antibody, anti-topoisomerase I antibody) manifestations. Data were analysed based on the presence of a low (= 20) or a high TSS (> 20) by both methods. Logistic regression analysis was performed to determine the independent variables that influenced TSS (in or by both methods) and death (after a 5-year follow-up). RESULTS: There was statistical association between TSS = 20 and limited SSc (both methods in the SSc model of two subgroups), calcinosis (MR-TSS) and anticentromere antibody (OR-TSS, trend). TSS > 20 was associated with diffuse SSc (both methods in the SSc models of two and three subgroups), pigmentary abnormalities (both methods), articular involvement (both methods), pulmonary restrictive disease (MR-TSS, trend) and anti-topoisomerase I antibody (OR-TSS, trend). Multivariate logistic regression analysis indicated that the SSc clinical variant was the most important factor that influenced significantly TSS (in both methods). After a 5-year follow-up, there were 20 deaths (35.7%); multivariate logistic regression analysis indicated that vascular involvement influenced death significantly, despite the TSS score. CONCLUSIONS: TSS, by both original and modified Rodnan methods, permits to differentiate distinct patterns in the clinical spectrum of SSc. In the model of two subgroups, there is a clear distinction between diffuse and limited disease, whereas in the model of three subgroups this distinction is not so clearly observed between the variants.
systemic sclerosis; skin score; skin thickness
