MUCOCUTANEOUS SYNDROME IN THE MAXILLOFACIAL AREA IN CHILDREN WITH SYSTEMIC SKLERODERMA

Systemic scleroderma (SSD) is a rapidly progressing disease with typical changes of the skin in the form of induration and atrophy of locomotor system, internal with fibrosive sclerotic processes in the heart and widespread vascular abnormalities similar to Reynaud’s syndrome, in which underlies a conjunctive tissue`s damage with fibrosis` predominance and vessels in the form of obliterating endarteritis. Among children this disease can arise at any age, but more often under 10 years old, girls are sick 5 times more often than boys.

The purpose of the research was to identify the main nosological signs of damage to the oral mucosa in children with systemic scleroderma.

Materials and methods of the research: we examined 38 children with diffuse scleroderma and carried out a histomorphologic examination of mucosa.

Results: a typical affection of mouth cavity`s mucous membrane is noted having a diffuse scleroderma. The children complain mainly of difficulties when opening the mouth, dryness, numbness of certain areas of the maxillofacial region, burning sensation, taste diversion. Mucousa of the mouth cavity endures 3 phases of main disease`s development (non-pitting edema, induration, atrophy). In the beginning we can see such changes of a mouth cavity`s mucous membrane as edema, mild hyperemia with obvious vascular pattern, with preserved compliance, sometimes elements of affection, anabrosis, aphthous stomatitis are among common symptoms. If the disease lasts for more than 2 years we can observe the phase of solid edema of mucous membrane turning gradually into the phase of induration when the mucosa is indurated and anematized. Histologic researches of gingival tissue confirm the clinical picture of damage, in 63,3 % determine dystrophy of epithelium, infiltration of epithelium by lymphocytes occurs in about 60%, reduction of blood stream is discovered  in 81,8%. 

Conclusion: the degree of mouth cavity`s mucosa affected in children with diffuse scleroderma depends on the form and duration of the main disease and on complication of baseline therapy which is confirmed by histomorphologic researches of mucous membrane.

Key words: Maxillofacial, Systemic Skleroderma