ABSTRACT Localized scleroderma (also called morphea) is a term encompassing a spectrum of sclerotic autoimmune diseases that primarily affect the skin, but also might involve underlying structures such as the fat, fascia, muscle, and bones. Its exact pathogenesis is still unknown, but several trigger factors in genetically predisposed individuals might initially lead to an immunologically triggered release of pro-inflammatory cytokines, resulting in a profound dysregulation of the connective tissue metabolism and ultimately to induction of fibrosis. To date, there are no specific serological markers available for localized scleroderma. Within the last years, several validated clinical scores have been introduced as potential outcome measures for the disease. Given the rarity of localized scleroderma, only few evidence-based therapeutical treatment options exist. So far, the most robust data is available for ultraviolet A1 phototherapy in disease that is restricted to the skin, and methotrexate alone or in combination with systemic corticosteroids in more severe disease that additionally affects extracutaneous structures. This practical review summarizes relevant information on the epidemiology, pathogenesis, clinical subtypes and classifications, differential diagnoses, clinical scores and outcome measures, and current treatment strategies of localized scleroderma.
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ABSTRACT: Background Localized Scleroderma (LoS) is characterized by a phase of disease activity followed by remission. However, disease recurrences occur.. Knowledge concerning these recurrences can help prompt treatment thereby preventing disease damage to develop.Objectives To investigate the frequency and characteristics of disease recurrences in pediatric and adult-onset LoS, and to identify patient variables that are associated with a higher risk of disease recurrence.Methods Retrospective chart reviews were performed of patients with LoS.Data concerning the frequency and characteristics of the disease recurrences were collected. A multivariate analysis was performed to identify patient variables that were associated with a higher risk of disease recurrence.ResultsA total of 344 patients were included in the analysis, of which 119 (35%) had pediatric-onset LoS and 225 patients (65%) had adult-onset LoS. Disease recurrence was present in 27% (n=32) of the pediatric-onset group and 17% (n=39) of the adult-onset group (p=0.037). Multivariate analysis identified a statistically significant association between disease recurrence and the linear LoS of the limbs subtype, independent of age at disease onset.Conclusions Recurrences in LoS occurred in almost a quarter of the patients and were most frequent in the linear LoS of the limbs subtype, independent of age at disease onset. These disease recurrences can occur even after many years of quiescent disease. Awareness of the high recurrence rates may help treating physicians to adequately recognize reactivation of the disease leading to a decreased delay in treatment reinitiation.This article is protected by copyright. All rights reserved.British Journal of Dermatology 11/2014; 172(3). DOI:10.1111/bjd.13514 · 4.10 Impact Factor
01/2013; 16:114. DOI:10.11604/pamj.2013.16.114.3065
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ABSTRACT: Morphea (localized scleroderma) is a rare fibrosing disorder of the skin and underlying tissues characterized by skin thickening and hardening due to increased collagen deposition. The significance of the disease depends on the extent of the lesions, potential for functional disability or cosmetic disfigurement, and presence or absence of extracutaneous manifestations. Treatment is tailored; circumscribed forms may require only topical treatment, while forms causing functional impairment or severe cosmetic change may require systemic treatment. Although localized scleroderma has a good prognosis, some clinical subtypes can be deforming and irreversibly disabling, especially when affecting the extremities or the face.Pediatric Clinics of North America 04/2014; 61(2):309-319. DOI:10.1016/j.pcl.2013.11.006 · 2.20 Impact Factor