From the Guidelines
The management of systemic sclerosis patients presenting with salt and pepper skin should prioritize immunosuppressive therapy and symptomatic relief, as outlined in the latest EULAR recommendations for the treatment of systemic sclerosis, updated in 2023 1. The salt and pepper skin appearance in systemic sclerosis is a result of the disease's autoimmune and fibrotic processes, leading to melanocyte damage and vascular changes.
- Key aspects of management include:
- Immunomodulation with medications such as methotrexate or mycophenolate mofetil to control the autoimmune component of the disease
- Symptomatic relief through skin care, including moisturizing and sun protection to prevent further skin damage
- Monitoring for and managing systemic complications, which are the primary drivers of morbidity and mortality in systemic sclerosis Given the high morbidity and mortality associated with systemic sclerosis, despite recent improvements in survival 1, it is crucial to follow the most current guidelines for management.
- The European Alliance of Associations for Rheumatology (EULAR) recommendations provide a comprehensive approach to the pharmacological management of systemic sclerosis, emphasizing the importance of early intervention and tailored treatment plans based on the extent of skin and visceral involvement 1. Treatment should be individualized, focusing on controlling disease progression and preventing systemic complications, rather than solely addressing the salt and pepper skin appearance, which is a manifestation of the underlying disease process 1.
From the Research
Salt and Pepper Skin in Systemic Sclerosis
- Salt and pepper skin is a manifestation of systemic sclerosis, characterized by diffuse hyperpigmentation with sparing of the perifollicular areas, resulting in a salt-and-pepper appearance 2.
- The exact mechanism of salt and pepper pigmentation is not fully understood, but it may be due to the richer capillary network around hair follicles, which preserves melanogenesis and produces perifollicular pigment retention 2.
- Salt and pepper pigmentation can be seen in various areas of the body, including the upper back, forearm, and legs, and may be associated with other skin manifestations such as thickening, hyperpigmentation, and tightening of the skin 2.
Management Approach for Systemic Sclerosis Patients with Salt and Pepper Skin
- There is no specific treatment for salt and pepper pigmentation in systemic sclerosis, but management of the underlying disease is crucial 3.
- Immunosuppressive therapies such as cyclophosphamide, mycophenolate mofetil, azathioprine, and methotrexate may be used to treat systemic sclerosis-related interstitial lung disease, which is a common complication of the disease 4.
- The choice of immunosuppressive therapy depends on the individual patient's condition and the severity of the disease, and may involve a combination of therapies 4.
- Early diagnosis and treatment of pulmonary arterial hypertension, a frequent and severe complication of systemic sclerosis, are highly challenging and require referral to an expert center 5.
Unusual Observations in Salt and Pepper Pigmentation
- In some cases, salt and pepper pigmentation may spare the skin creases and folds, which is an unusual observation that has not been previously reported 6.
- This sparing phenomenon may be related to the preservation of pigmentation over the superficial veins and the richer capillary network around hair follicles 6.