ORAL MANIFESTATIONS IN JUVENILE SCLERODERMA: CLINICAL PRESENTATIONS AND HISTOPATHOLOGICAL CHARACTERISTICS

Area of Science:

• Oral pathology
• Pediatric rheumatology
• Connective tissue diseases

Background:

• Juvenile scleroderma (JS) is a rare chronic connective tissue disorder affecting skin, soft tissues, and potentially internal organs.
• Oral cavity involvement in JS can lead to significant functional and cosmetic issues, impacting early childhood development.

Purpose of the Study:

• To investigate the histomorphological features of oral mucosa lesions in children diagnosed with juvenile scleroderma.
• To characterize the specific pathological changes and their prevalence in oral tissues of JS patients.

Main Methods:

• Dental examination and morphological investigation of oral mucosa in 39 juvenile scleroderma patients (9 with JSS, 20 with JSH).
• Analysis included histopathological assessment of tissue damage, vascularization, and immune complex deposition.

Main Results:

• Oral mucosa damage was observed in 100% of JS patients, manifesting early in the disease course.
• Histomorphological findings included dystrophic and atrophic processes, abnormal vascularization, and immune complex deposition (C3, IgM, IgG) in vessel walls (72.63%).
• Oral vasculitis (33.3%) and vasculopathy (52.63%) were prevalent; four stages of mucosal lesions were identified, with secondary infectious stomatitis noted.

Conclusions:

• Oral mucosa abnormalities, including dystrophic, atrophic, and vascular changes, are a consistent feature of juvenile scleroderma (JSS and JSH).
• The early onset and progressive nature of these oral lesions underscore the importance of early diagnosis and management in pediatric patients with JS.