Clinical improvement of salt-and-pepper skin changes in juvenile systemic sclerosis using mycophenolate mofetil after intravenous methylprednisolone: a 3-year follow-up.

In adult systemic sclerosis (SSc), salt-and-pepper skin changes can be used to diagnose diffuse cutaneous SSc during the early stages. However, reports of juvenile SSc (JSSc) with salt-and-pepper skin changes are unavailable. A 12-year-old Japanese girl presented with JSSc, showing scleroderma, Raynaud's phenomenon, digital ulcers, and telangiectasia. She developed scleroderma at 10 years of age and later experienced salt-and-pepper skin changes. Laboratory findings revealed positive antinuclear and anti-U3-RNP antibodies. After being diagnosed with JSSc based on established criteria, she received two courses of intravenous methylprednisolone (IVMP) followed by mycophenolate mofetil (MMF). Her modified Rodnan skin score improved from 25 to 0, and the salt-and-pepper changes resolved. This case represents the first report of the presence and subsequent improvement of salt-and-pepper skin changes in JSSc treated with MMF following IVMP. Recognition of salt-and-pepper changes may serve as an early clinical clue, prompting further diagnostic evaluation for JSSc and supporting early diagnosis of adult SSc. Moreover, MMF after IVMP may exert beneficial anti-fibrotic effects, potentially improving pigment changes by controlling scleroderma.