Localized scleroderma: a review

Abstract

Localized scleroderma (LoS) comprises a heterogeneous spectrum of fibrotic diseases that primarily affect the skin with inflammation and skin thickening. The extent of skin involvement and manifestation at extra-cutaneous structures characterizes the specific subset. Fat tissue, fascia, and sometimes muscle, and bones might be involved, and in rare cases the central nervous or ocular system. A worldwide accepted classification system for LoS is still lacking. The latest guidelines have proposed a distinction of limited, generalized, linear, deep, and mixed types. The exact pathophysiology of LoS is not fully understood yet, but it is thought that an externally triggered release of pro-inflammatory cytokines leads to a dysregulation of the connective tissue metabolism. LoS is diagnosed according to typical clinical features. Further diagnostic procedures are helpful to characterize the subtype and to define the extent of inflammation and fibrosis. Due to the heterogeneity of LoS, therapeutic strategies should always be selected depending on the respective subtype. The stage of tissue inflammation and sclerosis has to be considered. Treatment options such as topical corticosteroids, calcipotriol, and calcineurin inhibitors as well as phototherapy are well established. In subsets with severe wide-spread skin manifestation and/or extra-cutaneous involvement, methotrexate monotherapy or a combination of methotrexate and systemic corticosteroids need to be considered. Physical treatment should always be added to the topical and systemic treatment to prevent significant disabilities due to LoS. This article provides an updated review on the epidemiology, pathophysiology, clinical classification and treatment options for patients suffering from LoS.