Internal involvement in localized scleroderma

We studied 76 consecutive patients with localized scleroderma (morphea with or without linear scleroderma) and analyzed the frequency, prognosis, and predictors of internal involvement in a subset of 53 patients systematically investigated for the presence of such involvement. Internal involvement was found by systematic examination in 16 patients. Only 2 of them, including 1 patient who developed systemic scleroderma, had symptomatic and severe visceral disease. The other 14 patients had asymptomatic and minor abnormalities consisting of abnormal lower sphincter pressure, and/or peristaltic failure in the esophagus and slightly impaired carbon monoxide diffusion in the lung. Frequent seroimmunologic abnormalities had no predictive value. Three parameters were found to be associated with internal involvement: male gender (p < 0.05), increasing number of plaque-like lesions (p = 0.02) and hypergamma-globulinemia at 1st examination (p < 0.005). Mild esophageal and pulmonary involvement are not rare in morphea but are usually silent. In our series, after a median follow-up of 48 months, such involvement did not impair the prognosis. The mildness of these visceral abnormalities suggests that they do not justify routine detection in asymptomatic patients. Morphea and systemic scleroderma behave as 2 different diseases.