36º Congresso Brasileiro de Reumatologia

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Title

JUVENILE SCLERODERMA: SURVEY OF CASE.

Background

Scleroderma is rare-chronic, inflammatory disease. It is probably autoimmune, as well as featured by increasing of the collagen fiber synthesis and deposition which may result in fibrosis, in thickening of the skin and involvement of internal organ. It also may be localized and systemic. In addition it is estimated occurrence of three cases per year for one hundred thousand of people, and the localized scleroderma being the most common among children and teenagers. In the localized scleroderma the disease is narrow to the skin involvement and subcutaneous tissue, in some cases there may be involvement of muscle and bone. In the systemic form, besides of involvement of the cutaneous involvement, internal organs are also involved and different symptoms may occur according to harmed organ.

Case report

Patient is 13 years old has ulcer for two years in knees and thickening of tissues in extensor areas of knees and elbows, besides of weakness of proximal muscle of lower and upper limbs She related that had made a big effort with dyspnea and in the last months it was decreasing to the minimum effort. In physical test, she showed hardened skin mainly in hands, face, knees and elbows with ulcerous lesions. She also denies difficulties eating solid foods. Furthermore, additional examination pointed out pulmonary auscultation with slim crackles on base both of lungs mainly. Echocardiogram without changes, Antinuclear Factor (FAN) 1:640. Treatment with prednisone, reuquinol and hydroxychloroquine, and following up in the rheumatology outpatient clinic.

Conclusion

Then, once scleroderma is installed, it causes inflammation and production of fibrous tissue. The diagnosis is complex and treatment is based on each manifestation. The pathogenesis of scleroderma is complex, demanding interventions for minimizing the damages and promoting improvements on life quality, in face to that scleroderma may result from minimal to systemic aggressions, with several degrees of human commitment.

Área

Pediatrics

Autores

Éliton Carlos Batista de Sousa, Loisláyne Barros Leal, Sharlla Layana Leite Mendes, Debhora Geny De Sousa Costa, Benjamin Coêlho Lustosa de Araújo