Salt-and-pepper dyspigmentation as a distinctive feature in systemic sclerosis: A case series

Systemic sclerosis (SSc) is a rare autoimmune disorder characterized by tissue fibrosis, immune dysregulation, and vascular damage. SSc classically presents cutaneous features such as scleroderma, Raynaud’s phenomenon, salt-and-pepper dyspigmentation, and telangiectasia. Anticentromere, anti-Scl-70, and anti-RNA III antibodies are well-established biomarkers of SSc. This study aimed to illustrate the clinical profile of SSc patients from western Assam in northeast India. The American College of Rheumatology/European League Against Rheumatism classification criteria of 2013 were used for diagnosing SSc. Arthralgia, myopathy, and a history of Raynaud’s phenomenon were commonly observed, whereas sclerodactyly and puffy fingers were observed in seven and three cases, respectively. Half of the cases were found to have interstitial lung disease (ILD). This case series identified salt-and-pepper skin dyspigmentation as a distinctive cutaneous feature of SSc. One case also exhibited a unique presentation of serpentine supravenous hyperpigmentation (SSH) along the paths of superficial veins, against a backdrop of salt-and-pepper dyspigmentation. All the cases were found to be positive for antinuclear antibody using indirect immunofluorescence assay and for anti-Scl-70 antibody using line immunoassay (LIA). This study highlights salt-and-pepper dyspigmentation as a distinctive clinical feature of SSc, alongside anti-Scl-70 antibody positivity in all cases. Further research could provide a comprehensive explanation of these findings.