Abstract
Localized scleroderma is a rare, multifactorial, autoimmune disease of a chronic nature, typically without involvement of internal organs. Localized scleroderma is characterized by overproduction of collagen and increased deposition of the extracellular matrix.
We distinguish several subtypes, whereas the most common variant in adults is morphea, which typically affects the skin, while in children the linear form of localized scleroderma with deeper connective tissue involvement predominates. For the correct diagnosis of the disease, we rely primarily on the clinical picture and the characteristic histological findings.
Specific serologic markers have not been identified yet. The extent of the impairment of deeper subtypes is verified by imaging methods.
Therapeutically, topical corticosteroid or immunomodulatory externa are used in superficial localized scleroderma as a monotherapy or in combination with phototherapy (UVA/UVB). For deeper forms, we choose systemic treatment with methotrexate or in combination with systemic corticosteroids; in resistant cases, mycophenolate mofetil is a promising option.
The prognosis depends on the form of localized scleroderma , especially in deeper subtypes it is accompanied by mutilation and subsequent significant psychosocial impact, although without shortening the overall life expectancy.