title: [Scleroderma in children: a retrospective study of 70 cases].
abstract: BACKGROUND
  Scleroderma is uncommon in childhood. The aim of our study was to analyze the frequency of different clinical forms, their prognostic significance, biological features, and co-morbidities and to assess the pertinence of therapeutic options.
  
  
  METHODS
  The files of 70 children with primary scleroderma seen from 1980 to 1997 were retrospectively reviewed.
  
  
  RESULTS
  Localized scleroderma was observed in 56 children and diffuse lesions 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). Mean 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 antinuclear antibodies were found in 28 p. 100. Local corticosteroid therapy (level I or II) appeared to be useful in the superficial and active lesions (morphea) but did not halt progression to deep scleroderma. General corticosteroid therapy (1 mg/kg/24 h) 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 tissue disease) (6 cases), or scleroderma after eosinophil fasciitis (2 cases). Age at onset was around 9 years with female predominance. A particular gloves and socks form was observed and cardiac involvement was common, but there was no case of renal involvement. The therapeutic problems were similar to those in adults.
  
  
  CONCLUSIONS
  Our findings emphasize that scleroderma occurs readily in childhood, unlike what has been reported 10 years ago. Prognosis depends on functional impairment resulting from major sequelae particularly important in localized forms and the life-threatening situations occurring in systemic forms.
authors.affiliation: Service de Dermatologie, Groupe hospitalier Necker-Enfants Malades, Paris.
authors.forename: C
authors.initials: C
authors.lastname: Bodemer
authors.forename: M
authors.initials: M
authors.lastname: Belon
authors.forename: D
authors.initials: D
authors.lastname: Hamel-Teillac
authors.forename: J C
authors.initials: JC
authors.lastname: Amoric
authors.forename: S
authors.initials: S
authors.lastname: Fraitag
authors.forename: A M
authors.initials: AM
authors.lastname: Prieur
authors.forename: Y
authors.initials: Y
authors.lastname: De Prost
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000367:Age Factors; D017668:Age of Onset; D000974:Antibodies, Antinuclear / Q000032:analysis; D002648:Child; D015897:Comorbidity; D003882:Dermatomyositis / Q000453:epidemiology; D018450:Disease Progression; D004802:Eosinophilia / Q000453:epidemiology; D005208:Fasciitis / Q000453:epidemiology; D005260:Female; D006801:Humans; D008297:Male; D008947:Mixed Connective Tissue Disease / Q000453:epidemiology; D010297:Paris / Q000453:epidemiology; D011379:Prognosis; D012189:Retrospective Studies; D012594:Scleroderma, Localized / Q000453:epidemiology*; D012595:Scleroderma, Systemic / Q000453:epidemiology*; D012737:Sex Factors; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D000974:Antibodies, Antinuclear