Classification of juvenile systemic scleroderma
Juvenile systemic scleroderma, in which, apart from the widespread skin lesion, internal organs are involved in the process, it should be distinguished from juvenile and limited scleroderma, which is characterized by the development of sclerosis of the skin and underlying tissues without affecting the vessels and internal organs, although these diseases are often combined with the common term "juvenile scleroderma" .
There is no classification of juvenile systemic scleroderma, therefore a classification developed for adult patients is used.
Clinical forms of systemic scleroderma
- Prescleroderma. It can be diagnosed in a child with Raynaud's syndrome in the presence of specific antibodies - AT Scl-70, antibodies to the centromere (in the following, in most cases, juvenile systemic scleroderma develops).
- Systemic scleroderma with diffuse skin lesions (diffuse form) - rapidly progressive widespread skin lesions of the proximal and distal parts of the limbs, face, trunk and early defeat (within the first year) of internal organs, detection of antibodies to topoisomerase I (Scl-70).
- Systemic scleroderma with limited skin lesion (acrosclerotic form) is a long isolated Raynaud's syndrome, which precedes limited skin lesions of the distal forearms and hands, legs and feet, late visceral changes, detection of antibodies to the centromere. The peculiarity of juvenile systemic scleroderma is an atypical skin syndrome in the form of focal or linear (on hemitip) skin lesions, which is not a classical acrosclerotic variant.
- Scleroderma without scleroderma - visceral forms, in which the clinical picture is dominated by the defeat of internal organs and Reynaud's syndrome, and skin changes are minimal or absent.
- Cross forms are a combination of signs of systemic scleroderma and other systemic diseases of connective tissue or juvenile rheumatoid arthritis.
The course of systemic scleroderma is acute, subacute and chronic.
Degrees of disease activity: I - minimal, II - moderate and III - maximum. Determination of the degree of activity of systemic scleroderma is conditional and is based on clinical data - the severity of clinical symptoms, the prevalence of the lesion and the rate of progression of the disease.
Stages of systemic scleroderma:
- I - initial, identify 1-3 localization of the disease;
- II - generalization, reflects the systemic, polysyndromic nature of the disease;
- III - late (terminal), there is a failure of the function of one or more organs.
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