A comparative study of Limited Cutaneous Systemic Sclerosis versus diffuse cutaneous systemic sclerosis

Introduction: Systemic Sclerosis (SSc) is a multi-system connective tissue disorder of unknown aetiology characterised by thickening and stiffening of skin. SSc is clinically classified as limited cutaneous systemic sclerosis (LCSSc) and diffuse cutaneous systemic sclerosis (DCSSc). Objective: To compare the cutaneous and systemic features of LCSSc with those of DCSSc. Materials and Methods: A cross-sectional, descriptive study was conducted over a period of eight years in a tertiary care centre. All the patients of SSc were classified as LCSSc and DCSSc. Cutaneous features, systemic features and laboratory parameters were evaluated and compared between these two groups. Results: 54 cases of SSc were recruited for the study; of which 18 belonged to LCSSc and 36 to DCSSc. Female to male ratio was 5:1 in LCSSc and 11:1 in DCSSc. Mean age were 26.10±10.68 years and 34.72±15.95 years in LCSSc and DCSSc respectively. Statistically significant difference was observed in parameters like mean age, duration of Raynaud’s phenomenon, digital pitted scars, microstomia, diffuse hyperpigmentation, salt and pepper pigmentation, dyspnea on exertion, arthralgia, raised ESR and abnormality in spirometry. Conclusion: The duration of Raynaud’s phenomenon and digital pitted scars were significantly higher in LCSSc than DCSSc whereas microstomia, diffuse hyperpigmentation, salt and pepper pigmentation, dyspnea on exertion, arthragia, raised ESR and restrictive ventilatory defect were significantly higher in DCSSc than LCSSc. Pulmonary, renal and cardiac involvement were relatively less in our study population compared to other studies. Keywords: Limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis, Raynaud’s phenomenon, icrostomia, digital pitted scars, salt and pepper pigmentation.