Update on morphea

Abstract

Morphea is a rare fibrosing disorder of the skin and underlying tissues. The underlying pathogenesis of morphea is not completely understood at this time, but ultimately results in an imbalance of collagen production and destruction. Evidence-based treatment options of morphea are limited secondary to the rarity of the disease, and the lack of universally used validated outcome measures. The most commonly used outcome measures are skin scores, computerized surface area measurement, durometer, cutometer, thermography, and ultrasound measurements. The Localized Scleroderma Cutaneous Assessment Tool is a promising recently validated skin scoring tool that allows differentiation between activity and damage, is sensitive to change, and requires no additional equipment. The most robust data in the treatment of morphea exists for methotrexate in combination with systemic steroids and ultraviolet A1.

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

“…The first classification of LoSc was proposed in 1995 by Peterson et al (Mayo Clinic Classification) [8], who distinguished five main forms of the disease: plaque morphea, generalized morphea, bullous morphea, linear morphea and deep morphea (Table 1) [8]. The classification also included lichen sclerosus, eosinophilic fasciitis and atrophoderma of Pasini and Pierini [9,10]. A controversial aspect of the classification system was the fact that it excluded mixed scleroderma types which occur in almost 15% of patients [9].…”

“…The classification also included lichen sclerosus, eosinophilic fasciitis and atrophoderma of Pasini and Pierini [9,10]. A controversial aspect of the classification system was the fact that it excluded mixed scleroderma types which occur in almost 15% of patients [9]. In…”

“…1) [8]. Wspomniana klasyfikacja obejmowała także: liszaj twardzinowy, eozynofilowe zapalenie powięzi oraz twardzinę ograniczoną pierwotnie zanikową [9,10]. Dyskusyjne w tych klasyfikacjach było nieuwzględnienie postaci mieszanych twardziny, które występują u blisko Limited form of localized scleroderma…”

“…• Generalized localized scleroderma -variety diagnosed in 7-9% of patients, characterized by ≥ 4 areas of skin induration, ≥ 3 cm in diameter, in ≥ 2 anatomic sites [2,9]. 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.…”

“…• Twardzina ograniczona uogólniona -odmiana stwierdzana u 7-9% chorych, cechuje się występo-waniem ≥ 4 ognisk stwardnienia o średnicy ≥ 3 cm, zajmujących ≥ 2 obszary anatomiczne [2,9]. Zmiany lokalizują się symetrycznie w obrębie tułowia, ud i okolicy krzyżowo-lędźwiowej [2,6].…”