SCLERODERMA IN THE CHILDHOOD PERIOD

¹Erguven M, ²Hatipoglu S, ¹Katıoz Y, ¹Cakır B, ¹ Usta M, ¹Ozcay S

1 SSK Goztepe, Department of Pediatrics, Education Hospital,

2 Dr. Sadi Konuk Department of Pediatrics, Education Hospital, Istanbul, Turkey

Scleroderma is an autoimmune disease characterized by excess deposition of collagenous material in the cutaneous tissuse, lung, heart and gastrointestinal system. Thickening and fibrozis, of the tissues cause dysfunction and failure of the body system. Sınce it's rare in the childhood period, we reported three cases representing local and diffuse forms of the disease. First case who's 10 years old girl, presented with only thickening of the skin on her lower extremity without any systemic involvement, has a favorite prognosis. But the other two cases, a 13 years old boy and a 12 years old girl have diffuse form due to the gastrointestional system involvement besides cutaneus manifestations of the disease. Skin, epidermis and muscle tissues of the trunk in the second case and lower extremity ınvolvement in the third case were present. Both cases had esophagial involvement diagnosed according to barium meal, endoscopy and pH monitorization. Antibodies directed against scl-70 were high in the first case. D-penisillamine 150 mg per day and deflasakort 10 mg per twice a day is administered to all the patients as the medical treatment. And all patients are taken to physical therapy programme to control the complications in the long run. Scleroderma cases are diagnosed rarely in childhood and localized form of scleroderma may devolop to systemic form. These patients must be followed with multidiciplinary approachment.

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