Background: Juvenile scleroderma is a rarely seen chronic connective tissue disorder
characterized by stiffening of the skin. The frequency of the disease was reported as one per million.
According to organ involvement, the disease is divided into two main forms: systemic and localized
Objective: Since it is uncommon in children, many aspects of the disease remain discussable. With
this review, we aimed to revise recent findings and new developments in this rare condition.
Method: A systematic literature research was performed, using the following medical databases:
Pubmed/ Medline and the Cochrane Library. We searched for up-to-date randomized controlled
studies, case-control studies, and cohort studies and cases reports on juvenile scleroderma (both
systemic and localized form).
Results: Skin manifestations are most prominent features of the systemic form, followed by musculoskeletal
and vascular involvement. Cardiovascular, gastrointestinal and renal disorders are rare in
childhood. Combination of disease modifying anti- rheumatic drugs (methotrexate, mycophenolatemofetil,
cyclosporine) and steroid reprents the first line therapy. Bosentan is used for cases with
pulmonary hypertension and for extensive digital ulcerations. Biological treatment emerges as a
useful treatment option in most severe form of the disease. Localized scleroderma is characterized
with sclerodermatosis of the skin. Internal organ involvement is not expected. Classification of the
local scleroderma is made according to the size and localization of the skin changes. There are few
different therapeutical options but there is no specific therapy for the localized scleroderma.
Conclusion: Many data regarding disease features and treatment options in juvenile scleroderma
are based on studies among adults. There is a striking need for multicentric, prospective studies
among children with juvenile scleroderma, in order to elucidate some questions of clinical course
and disease prognosis. Recent genetic studies have revealed the role of the genetic factors (namely
HLA class II) in the pathogenesis of the disease. Emerging biological agents and new treatment options
are showing promising results.