Progressive systemic sclerosis is a disease of uncertain etiology associated with vascular/collagen tissue changes that may affect only the skin (scleroderma) or any and all internal organs (PSS). A disease primarily of Caucasian females, PSS is usually manifested in the fourth to sixth decade of life. Clinical and laboratory diagnostic testing is often confused by overlap phenomena, but recent research has increased diagnostic specificity. There is no known cure for PSS. Nevertheless, the knowledgeable clinician has an important role to play in the PSS patient's future. Symptom management and function maintenance will require an ongoing and understanding relationship between practitioner and patient.