Patients (4) with concurrent, chronic, progressive, localized scleroderma and discoid lupus erythematosus [LE] were studied; the condition originated as linear scleroderma in 3 of them. Three of the four patients were young females at the onset of the 1st skin disease. Dermatopathologic study confirmed the scleroderma and LE. Direct immunofluorescence showed a positive band test in 3 cases. Unusual serological results included a positive LE clot test in 3 cases, a positive extractable nuclear antigen test in 1 case and a negative anti-nuclear antibody test on repeated occasions in all 4 cases. Rare cutaneous disease similar to systemic, mixed or overlap connective tissue disease exists and offers an opportunity to study unusual immunologic and pathological events in scleroderma and LE.