Scleroderma in childhood: a retrospective study of 70 cases.

Background. Scleroderma is uncommon in childhood. The aim of our study was to analyze the frequency of different clinical forms, their prognostic sign ificance, biological features, and co-morbidities and to assess the pertine nce of therapeutic options. Patients and methods. The files of 70 children with primary scleroderma see n from 1980 to 1997 were retrospectively reviewed. Results. Localized scleroderma was observed in 56 children and diffuse lesi ons in 14. Localized scleroderma (44 girls, 12 boys) began early at a mean age of 7 years 2 months. The lesions presented as isolated bands (39 p. 100 ), associated with morphea (36 p. 100), or multiple morphea (5 p. 100). Mea n duration of the clinical course was longer in cases with more and deeper lesions. Eosinophilia was observed at onset in 38 p. 100 of the cases and a ntinuclear antibodies were found in 28 p. 100. Local corticosteroid therapy (level I or II) appeared to be useful in the superficial and active lesion s (morphea) but did not halt progression to deep scleroderma. General corti costeroid therapy (1 mg/kg/24h) did not prevent the development of sequelae in cases with bands (16/16). Diffuse scleroderma corresponded to systemic scleroderma (6 cases), dual morbidity (dermatomyositis, mixed connective ti ssue disease) (6 cases), or scleroderma after eosinophil fasciitis (2 cases ). Age at onset was around 9 years with female predominance. A particular g loves and socks form was observed and cardiac involvement was common, but t here was no case of renal involvement. The therapeutic problems were simila r to those in adults. Discussion. Our findings emphasize that scleroderma occurs readily in child hood, unlike what has been reported 10 years ago. Prognosis depends on func tional impairment resulting from major sequelae particularly important in l ocalized forms and the life-threatening situations occurring in systemic fo rms.