[Lesions of the hand and upper limb in children with linear scleroderma. Therapeutic approach in two cases]

The authors report two cases of linear scleroderma (LS) in children, located on the hand and upper limb. LS is rare in children (245 cases reported in the literature). LS initially consists of a sclero-inflammatory lesion, followed by a sclero-atrophic lesion, characterized by its linear arrangement selective involvement of one limb, and its local and regional effects. The onset is generally marked by development of a sclerodermic line, sometimes associated with extra-cutaneous lesions. The active phase of the disease is long (mean: 3 years), with multiplication or extension of the initial lesions (frontal "coup de sabre", hemiatrophy). Systemic complications are rare (Raynaud's phenomenon, glomerulonephritis...). Regional complications (retractile myositis, articular stiffness, shortening) are frequent, severe and persistent (75% of cases). Treatment has not been definitively standardized, but should include:--systemic corticosteroids, quite active in early forms. --physiotherapy and external prostheses during the early and active phase, and finally surgical correction of orthopaedic sequelae. They are often difficult operations, with partial results, but functionally very useful, especially with the addition of cutaneous expansion. Our 2 cases demonstrate the considerable functional improvement achieved in the hand.