In linear scleroderma, a rare form of circumscribed scleroderma, the lesions are arranged in a band-shaped linear distribution and both the superficial and the deeper layers of the skin are attached to the underlying structures. This disease must principally be differentiated from eosinophilic fasciitis (Shulman's syndrome). On the basis of the clinical and laboratory data, systemic scleroderma can be excluded in nearly all the cases. Nevertheless, patients with linear scleroderma might develop systemic scleroderma or other systemic diseases of the connective tissue even after years. As a consequence, thorough physical examination as well as laboratory evaluation is necessary over a long follow-up period. We introduce a new classification of circumscribed scleroderma.