Rapid Diffuse Skin Disease with Progressive Joint Contractures

Systemic sclerosis is a complex connective tissue disease with high morbidity and mortality. The scleroderma patient with rapidly progressive skin disease and joint contractures is one of the most challenging factors a clinician will face. It is important to recognize that such patients may be at increased risk of severe internal organ involvement. The timing for institution of therapies is important and may lead to improved outcomes if undertaken early. With the evolving therapeutic landscape, there is the promise for treatments that may prove to be effective in the treatment of diffuse cutaneous systemic sclerosis. This chapter highlights a systemic sclerosis patient with rapidly progressive skin disease and considerations for therapies.