Rapidly Progressive Skin Disease in a Patient with Diffuse Systemic Sclerosis

Abstract

Systemic sclerosis (Scleroderma, SSc) is a disease of unknown etiology characterized clinically by thickening of the skin and various degrees of internal organ fibrosis. The extent of skin involvement is instrumental in classifying patients within the SSc spectrum: diffuse, limited, or sine scleroderma. Traditionally, patients with diffuse scleroderma have a worse prognosis with the extent and speed of skin involvement correlating well with global disease severity. Particular attention needs to be paid to a distinct subgroup of SSc patients characterized by rapidly progressive skin involvement and anti-topoisomerase I antibodies since they are at an increased risk for end organ damage leading to higher mortality and cases with anti-RNA pol III that are at particular risk of scleroderma renal crisis. Skin involvement can be measured using the modified Rodnan skin score (MRSS). The current skin-focused therapies are of marginal efficacy at best, and disease modifying agents used to target the disease as a whole have unpredictable effects on the skin involvement. Knowledge of skin involvement in SSc is expanding as interventional clinical trials generally use skin biopsy findings and MRSS as outcome measures. When faced with a patient experiencing rapid involvement of new areas of the skin and worsening of the previously involved skin, the challenge is to monitor for new internal organ involvement while implementing measures to prevent long-term disability: weekly home blood pressure monitoring, vigorous occupational and physical therapy, pain relief and balanced nutrition.