Localized scleroderma

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.

Localized scleroderma – classification and tools used for the evaluation of tissue damage and disease activity/severity

“…Choroba klinicznie obejmuje trzy fazy: wczesną zapalną (aktywną, utrzymującą się średnio przez 3-4 lata), postę-pującego stwardnienia i zanikową (atroficzną) (ryc. 1) [5,6]. Twardzina ograniczona przeważnie nie ulega progresji do układowej postaci twardziny, chociaż są doniesienia o pojawianiu się LoSc w przebiegu twardziny układowej z ograniczonymi stwardnieniami skóry [2].…”

“…The clinical course of LoSc includes three stages: early inflammation (active stage persisting for an average of 3-4 years), progressive sclerosis and atrophy ( Fig. 1) [5,6]. In the majority of cases LoSc does not progress to systemic sclerosis, however there have been reports on the development of LoSc in the course of systemic sclerosis with limited scleroderma [2].…”

“…The lesions are distributed symmetrically within the trunk, thighs and in the lumbosacral region [2,6], and easily distinguishable from the surrounding healthy skin (Figs. 4 A-D) [1,3].…”

“…• Guttate localized morphea -characterized by multiple, yellow-white, small papules with a shiny surface, < 1 cm in diameter, located primarily on the trunk [2,4,6]. Clinically and histopathologically, they may imitate extragenital lichen sclerosus [6,10].…”

“…• Atrophoderma of Pasini and Pierini (APP) -regarded as an early abortive form of LoSc associated with a mild course [4,6]. It is manifested as oval lesions, greyish brown in colour, either flat or slightly depressed, without skin induration (Fig.…”