From the Guidelines
Salt and pepper skin in systemic sclerosis is caused by a combination of hyperpigmentation and hypopigmentation due to the underlying disease process, with no specific treatment targeting this pigmentation directly, and management focusing on controlling the underlying systemic sclerosis with immunosuppressive medications. This distinctive skin appearance develops when melanocytes, the cells responsible for skin pigmentation, are damaged or destroyed in some areas while melanin production increases in others 1. The hypopigmentation occurs when melanocytes are lost during the inflammatory and fibrotic processes of systemic sclerosis, particularly in areas where skin atrophy develops. Simultaneously, hyperpigmentation develops in surrounding areas due to increased melanin production triggered by inflammatory mediators and growth factors released during the disease.
Some key points to consider in the management of systemic sclerosis include:
- The use of immunosuppressive agents such as mycophenolate mofetil, methotrexate, cyclophosphamide, rituximab, and tocilizumab for the treatment of skin fibrosis and other disease manifestations 1
- The importance of early diagnosis and treatment to prevent long-term damage and improve quality of life 1
- The need for a multidisciplinary approach to management, including rheumatology, dermatology, and other specialties as needed 1
In terms of specific treatments, mycophenolate mofetil and nintedanib are recommended for the treatment of skin fibrosis, and rituximab and tocilizumab may also be considered 1. However, the most effective treatment approach will depend on the individual patient's disease characteristics and response to therapy. Overall, the goal of treatment is to control the underlying disease process and prevent long-term damage, rather than specifically targeting the salt and pepper skin appearance.
From the Research
Causes of Salt and Pepper Skin in Systemic Sclerosis
- The exact cause of salt and pepper skin in systemic sclerosis is not fully understood, but it is believed to be related to the disease's effects on the skin's pigmentation and blood vessels 2.
- One theory is that the richer capillary network in the perifollicular areas may warm the skin and preserve melanogenesis, resulting in the characteristic salt and pepper appearance 2.
- The destruction of melanocytes, which can be triggered by a combination of cellular and humoral immune factors, as well as external factors such as trauma or inflammation, may also contribute to the development of salt and pepper skin 2.
- Physical factors like temperature changes, as well as genetic and hormonal factors, may also influence pigment formation and contribute to the development of salt and pepper skin 2.
Characteristics of Salt and Pepper Skin in Systemic Sclerosis
- Salt and pepper skin in systemic sclerosis is characterized by a diffuse hyperpigmentation with sparing of the perifollicular areas, resulting in a salt and pepper appearance 3, 2.
- The pigmentation is often preserved over the superficial veins 3.
- In some cases, the salt and pepper pigmentation may spare the skin creases and folds, which is a relatively uncommon finding 3.
- The skin changes associated with systemic sclerosis, including salt and pepper skin, can be accompanied by other symptoms such as thickening, tightening, and induration of the skin, as well as difficulty swallowing and shortness of breath 2.