Juvenile systemic sclerosis

Abstract

Juvenile systemic sclerosis (jSSc) is a rare multisystem connective tissue disease. Approximately 5-10% of all adults with systemic sclerosis (SSc) report the onset of the disease during childhood. It has a variety of clinical manifestations, sometimes different from the adult form. Mixed connective tissue disease (MCTD) and overlap syndromes have features of jSSc and sometimes even fulfill the criteria of jSSc [1]. It is important to differentiate it from juvenile localized scleroderma, which is much more common in the pediatric population, it occurs in 4.7-20 per 100,000, and it is 10 times more frequent than jSSc. It has in most cases a more benign course with the exception of pansclerotic morphea [2]. Localized scleroderma rarely evolves into systemic sclerosis in childhood, although in adults it is well recognized that cases of systemic sclerosis may also demonstrate localized scleroderma.