Clinical and Dermoscopic Features of Pigmentary Changes in Scleroderma and Its Association with Systemic Involvement - A Descriptive Study from a Tertiary Care Centre in South India

Abstract Background: Systemic sclerosis (SSc) is a multisystem disease with the highest case-specific mortality among other autoimmune diseases. Dermatological manifestations include skin thickening, Raynaud’s phenomenon, digital ulcers, pitted scarring and pigmentary abnormalities. Vascular abnormalities in systemic sclerosis are responsible for various systemic complications including pulmonary hypertension and scleroderma renal crisis. The underlying vascular pathology responsible for organ involvement might also result in skin manifestations including Raynaud’s phenomenon and digital ulcers. However, vascular abnormalities resulting in pigmentary changes in systemic sclerosis have not been well studied. Pigmentary changes, which are more common among the darker phenotypes, may act as an early marker for the identification of patients at risk for subsequent organ damage. We aim to study various clinical and dermoscopic features of pigmentary abnormalities in systemic sclerosis and its association with systemic features. Materials and Methods: A cross-sectional study was conducted on 60 patients with scleroderma who attended the autoimmune clinic at the Department of Dermatology and Venereology Government Medical College, Kozhikode. Detailed clinical examination and investigations were performed for dermatological manifestations with special emphasis on pigmentary changes. Pigmentary changes were examined with the help of a digital dermoscope, and the different patterns observed were classified into pigmentary, vascular and scale patterns. Results: In the present study, a female predominance was observed. The mean age of the study group was 44 ± 13 years. Out of the 60 patients, 52 (86.7%) had limited cutaneous disease and 8 (13.3%) had diffuse cutaneous disease. The MRSS varied from 3 to 32 with a mean of 14.22 ± 5.76. The most common type of pigmentary change observed was vitiligo like macules (70%) followed by salt and pepper pigmentation (61.6%). Other types of pigmentation were localised diffuse hyperpigmentation, reticulate hyperpigmentation, localized hypopigmentation, melasma like hyperpigmentation and periorbital hyperpigmentation. On dermoscopy, the most common pigmentary pattern in vitiligo like macules was the petaloid pattern (57%). White homogenous areas and perifollicular pigment (89.2%) were the predominant findings in salt and pepper pigmentation. More than 50% of patients with salt and pepper pigmentation, melasma like and periorbital pigmentation and more than 25% of patients with vitiligo like macules and reticulate pigmentation had red structureless areas on dermoscopy. Only 10% of patients with localised diffuse hyperpigmentation had red structureless areas. About 70% of patients with vitiligo like macules, salt and pepper and localised diffuse hyperpigmentation had digital ulcers. Around 20% of patients with vitiligo like macules and salt and pepper pigmentation and 30% of patients with localised diffuse hyperpigmentation had interstitial lung disease. Limitations: Our study is limited by being institutional based and may not reflect the actual incidence of pigmentary disturbance in the general population with SSc. Conclusion: We have described various clinical and dermoscopic findings in all types of pigmentary changes in SSc. Dermoscopy of pigmentary changes may be as helpful as nail fold capillaroscopy in correct diagnosis and assessing the prognosis of the disease.