Scleroderma is a disease of unknown origin, characterized by thickening and induration of the skin, involving, in many cases, internal organs. Two main types are clinically observed: systemic sclerosis, a progressive and multisystemic disorder, and localized scleroderma (morphea), in which sclerosis is limited to the skin. Main subtypes of morphea are: circumscribed, guttate, linear, nodular and profunda. The commonest form of scleroderma in childhood is morphea, and among this, linear lesions are the most frequent. These can be divided in 4 subtypes. 1) lesions involving the limbs, 2) the form so called "en coupe de sabre" (of the face), 3) Parry-Romberg syndrome (hemifacial atrophy), and 4) disabling pansclerotic morphea. Polimorphic clinical features and subcutaneous involvement, with atrophy and hyperpigmentation, are more common in childhood. Actually, topical steroids represent the more effective therapy, particularly in early stages of the disease. Recent studies demonstrated that for its immunomodulating and antifibrotic properties calcitriol treatment may be also attempted with encouraging results.
|Translated title of the contribution||Localized scleroderma or morphea in childhood|
|Number of pages||6|
|Journal||Progressi in Reumatologia|
|Publication status||Published - 2003|
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