What happens to the hands and feet in systemic sclerosis (SSc)?

From the Guidelines

In scleroderma, the hands and feet are commonly affected by Raynaud's phenomenon, digital ulcers, and skin thickening, leading to significant morbidity and impact on quality of life. According to the most recent guidelines 1, the management of these symptoms involves a multifaceted approach, including lifestyle modifications, medications, and physical therapy. The hands and feet may develop Raynaud's phenomenon, characterized by episodes where fingers and toes turn white, then blue, and finally red in response to cold or stress, due to abnormal blood vessel constriction. Additionally, the skin on the hands and feet becomes thick, tight, and shiny (sclerodactyly), limiting mobility and causing pain.

• Key symptoms and manifestations include:
  • Raynaud's phenomenon
  • Digital ulcers
  • Sclerodactyly
  • Calcinosis
  • Contractures
  • Numbness or tingling from nerve compression
• Management strategies include:
  • Keeping extremities warm
  • Using medications like calcium channel blockers (nifedipine 30-60mg daily) or phosphodiesterase inhibitors (sildenafil 20mg three times daily) to improve circulation
  • Physical therapy to maintain hand function
  • Specialized treatments like intravenous prostacyclin analogs for severe digital ulcers These symptoms and management strategies are supported by the latest recommendations from the EULAR guidelines 1, which emphasize the importance of a comprehensive approach to managing scleroderma-related symptoms and improving quality of life.

From the Research

Scleroderma Effects on Hands and Feet

• Scleroderma can cause digital ulcers, which are ischemic ulcerations of the fingertips, leading to pain and disability 2, 3, 4, 5.
• These ulcers can be a source of significant morbidity, and their healing can be challenging due to the underlying vascular abnormalities and fibrosis associated with scleroderma 2, 3, 4, 5.
• In addition to digital ulcers, scleroderma can also cause other hand manifestations, including:
  • Calcium deposits within the soft tissues, which can cause pain and ulcerate through the skin 6.
  • Digital ischemia, resulting in chronic wounds and digital gangrene 6.
  • Joint contracture, leading to limited mobility and functionality of the hands 6.
• The feet can also be affected by scleroderma, although this is less commonly discussed in the literature.
• Treatment options for scleroderma-related digital ulcers include:
  • Pharmacologic therapies, such as bosentan, iloprost, and phosphodiesterase-5 inhibitors 2, 3, 4, 5.
  • Surgical intervention, which can be challenging due to the poorly vascularized tissue and deficient soft tissue envelope, but may be necessary to address disabling hand conditions 6.

Treatment Outcomes

• Studies have shown that iloprost can be an effective long-term treatment for scleroderma-related digital ulcers, achieving healing and prevention in a significant proportion of patients 5.
• Bosentan has also been shown to reduce the occurrence of new digital ulcers, although its effect on healing existing ulcers is less clear 2, 3.
• Phosphodiesterase-5 inhibitors have been found to be significant for digital ulcer healing, with a pooled risk ratio of 3.28 3.