[Systemic scleroderma in children. Apropos of 5 cases. A review of the literature].
Systemic scleroderma is a rare disease in childhood. 62 cases are analyzed. Cutaneous manifestations are identical to those seen in adults. However Raynaud phenomenon is much more frequently missing but follow-up of patients is only of 4 years' duration. We want to draw attention on possible worsening of clinical signs during intercurrent infectious episodes. We report exceptional roentgenological bone anomalies. Gastro-intestinal tract is frequently involved, particularly the oesophagus. We want to draw attention on latent small intestine involvement. A normal thoracic X-ray examination cannot rule out involvement of the lungs; systematic respiratory functional tests are absolutely necessary. All parts of the cardiac wall can be involved: we underline the particular seriousness of this involvement as it was exclusively responsible of death in 10 cases out of 21. Renal involvement is rare. We are reporting 2 cases where a staturo-ponderal retardation remains unexplained; 7 other cases in the literature report on isolated weight retardation. Biological anomalies are similar to those reported in adult. Treatment is not well-codified; we can hope that a better understanding of the disease's physiopathology will lead to the discovery of an efficient therapy.
D000293:Adolescent; D000328:Adult; D001842:Bone and Bones / Q000473:pathology; D002648:Child; D004064:Digestive System / Q000473:pathology; D005260:Female; D006801:Humans; D007596:Joints / Q000473:pathology; D007668:Kidney / Q000473:pathology; D008168:Lung / Q000473:pathology; D008297:Male; D009132:Muscles / Q000473:pathology; D009206:Myocardium / Q000473:pathology; D011928:Raynaud Disease / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D012595:Scleroderma, Systemic / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D012598:Sclerosis; D012867:Skin / Q000473:pathology; D014070:Tooth / Q000473:pathology
Sclérodermie systémique de l'enfant. A propos de 5 observations. Revue de la littérature.