Childhood-onset scleroderma: is it different from adult-onset disease

Abstract

Objective: To distinguish childhood-onset scleroderma from adult-onset disease.

Methods: The clinical and serologic features of 58 patients with childhood-onset scleroderma (11 patients with diffuse cutaneous systemic sclerosis [SSc], 16 with linear SSc, 14 with linear morphea, and 17 with morphea) were examined in the largest cohort of such patients studied to date. These parameters were compared with data obtained from patients with adult-onset disease.

Results: Childhood-onset scleroderma resembled adult-onset disease with regard to the heterogeneity of clinical expression and subsets of disease, but it also differed from adult-onset disease in a number of clinical and laboratory parameters. The predominant childhood-onset disease presentation was the localized form of the disease, with limited and diffuse SSc being less notable. There was a significant association of trauma with childhood-onset scleroderma (P < 0.0001), which was not noted in adult-onset disease. Furthermore, in contrast to adult disease, patients with childhood-onset disease had normal levels of parameters of vascular activation (von Willebrand factor, angiotensin-converting enzyme, E-selectin, and endothelin-1), T cell activation (soluble interleukin-2 receptors), and collagen synthesis (carboxy-terminal type I and amino-terminal type III), a notable lack of anticentromere antibodies, and abnormal coagulation indices.

Conclusion: A number of features distinguish childhood-onset scleroderma from adult-onset disease.