Juvenile localized scleroderma

Abstract

Juvenile localized scleroderma (JLS), also known as morphea, comprises a group of conditions in which the process of fibrosis involves essentially the skin and subcutaneous tissues. They may range from very small plaques to extensive indurate lesions which cause significant functional and cosmetic deformity. The most widely used classification divides JLS into five general types: plaque morphea, generalized morphea, bullous morphea, linear scleroderma, and deep morphea [1]. Some conditions, such as atrophoderma of Pasini and Pierini, eosinophilic fasciitis, or lichen sclerosus et atrophicus, are sometimes classified among the subtypes of JLS, but this aspect is still controversial. This classification does not include the mixed forms of JLS where different types of lesions occur in the same individual. This subtype is more common than previously recognized, accounting for 15% of the whole group [2]. A proposal for a new classification includes five subtypes: circumscribed morphea (CM), linear scleroderma, generalized morphea (GM), pansclerotic morphea, and the new mixed subtype where a combination of two or more of the previous subtypes is present (Table 9.1) [3].