[Generalized sclerosis (scleroderma) in children].

Five patients with childhood scleroderma, were studied from a total group of 50 cases with the disease, 39 of them with diffuse systemic sclerosis and 11 with the CREST syndrome. The average age for these five patients when the disease onset was 13 (the age ranged from 5.5 to 16 years) with an average follow-up of 3.6 years (ranging from 1 to 6.5 years). Of the five, four girls were classified as having diffuse systemic sclerosis and the remaining boy, as suffering from the CREST syndrome. We found no family history or personal and occupational antecendents related with the appearance of the illness. Also excluded were conditions associated with changes similar to scleroderma as are seen in cases of diabetes mellitus, phenylketonuria, toxic oil syndrome, or graft-host rejection reactions. The clinical manifestations seen at the start of the disease included the Raynaud phenomenon, subcutaneous edema and muscular-skeletal abnormalities as arthralgia and myalgia with objective data of inflammatory myopathy. Proximal scleroderma was seen in all five patients; three of them, in addition, developed rapidly progressive cutaneous changes, causing the loss of elasticity and cutaneous hardening of the face during the first year of the disease. In all of the cases, the skin biopsy showed histopathological changes compatible with the diagnosis already given. The most important changes seen in the organs of these children were oesophageal dysfunction and fibrosis of the lung. The X-rays of three of the patients showed them to suffer from intestinal malfunction. We found no kidney, liver or nervous system disorders.(ABSTRACT TRUNCATED AT 250 WORDS)