Juvenile localized scleroderma: clinical picture, diagnosis, therapeutic update (literature review and own observations)

Abstract

Among rheumatic diseases in children, a special place belongs to juvenile localized scleroderma, which is highlighted as a separate form in the structure of scleroderma diseases and is characterized by: the onset of the disease up to 16 years, skin lesions, often focal or linear (hemiforma), a tendency to form contractures and the development of limbs abnormalities. This form of the disease is characterized by damage to the skin mainly involving the surrounding muscles. The cause of juvenile localized scleroderma is unclear. Three main components underlie the development of the disease: damage to the vascular bed, activation of the immune system with a violation of its regulation and release of cytokines, as well as altered collagen synthesis with proliferation of fibroblasts and the subsequent development of fibrosis. Several forms of juvenile localized scleroderma have been identified, differing in clinical manifestations and depth of lesion of subjacent tissues, and among the skin manifestations, five subtypes are distinguished: plaque, generalized, linear, pansclerotic, and mixed. Diagnosis is based on an analysis of the history and typical clinical picture of the disease. Specific laboratory tests to confirm the disease has not been developed. Modern treatment principles for juvenile localized scleroderma involve the use of different groups of drugs, including methotrexate, corticosteroids, tacrolimus, cyclosporine, imatinibum, mycophenolate mofetil, retinoids, PUVA therapy (Psoralen + UltraViolet A), use of slow calcium channel antagonists (nifedipinum group), bosentan. The studies currently being conducted in the world on the standardization of basic therapy of juvenile localized scleroderma are likely to reveal the optimal variant and duration of treatment for each form of the disease and improve the prognosis of the disease. The article also presents the results of own clinical observations.