ORAL MANIFESTATIONS IN JUVENILE SCLERODERMA: CLINICAL PRESENTATIONS AND HISTOPATHOLOGICAL CHARACTERISTICS

Juvenile scleroderma (JS) is a rare chronic connective tissue disorder characterized by stiffening of the skin and soft tissues, including the oral cavity and perioral tissues, leading to fibrosis and a large spectrum of internal organs involvement, cosmetic defects, and early infant disability. The aim of this study was to investigate the histomorphological features of lesions of oral mucosa tissues in children with juvenile scleroderma (JS). 39 JS patients (9 with juvenile systemic sclerosis - JSS and 20 with juvenile scleroderma of head-JSH aged from 5 to 17 years were observed with dental examination and morphological investigation of the dental mucosa. The oral mucosa in patients with JS showed signs of damage of the mucous membrane of the oral cavity, was detected in 100% of JS patients observed. Lesions of mucosa appear at the earliest stages of the disease and are associated with the development of dystrophic and atrophic processes, and abnormal vascularization. In 72.63% of patients, C3 deposition of the complement component and IgM and IgG containing immune complexes were found in the vessel walls of the oral cavity. Vasculitis of the oral cavity was detected among 1/3 of patients, and vasculopathy was found among 52,63 % of those examined. Mucosal, dystrophic, and vascular abnormalities develop in children with JSS, as well as in JSH patients. We distinguish and describe four stages of mucous lesions. Secondary infection stomatitis was noticed in patients.