Morphea: a practical review of its diagnosis, classification and treatment

Abstract

Morphea, or localized scleroderma, is a rare disease of the connective tissue that manifests itself with localized sclerosis of the skin and, in some cases, with extracutaneous manifestations. Its etiology is not fully understood, but it is believed that there is genetic predisposition, in addition to environmental triggering factors. Classification of the disease is not simple due to its multiple presentations; however, it is useful in order to define the treatment, which should be individualized and started early to avoid cosmetic and functional complications. In this review, we summarize the most important practical aspects of the classification, diagnostic methods and evaluation of morphea activity, as well as available therapeutic options, with an emphasis on existing clinical evidence regarding their efficacy and safety.

Comparison of the tissue expressions of glutathione S transferase isoenzymes among patients with morphea and healthy controls: A preliminary study

“…6 Decreased antioxidant capacity such as lower levels of serum alpha-tocopherol, selenium, carotene and ascorbic acid, and higher values of oxidant molecules contribute to the oxidative stress mechanism in scleroderma pathogenesis. 3 In in-vitro studies it has been shown that fibroblasts cultured from scleroderma lesions, release increased amounts of reactive oxidizing species which on the other hand induce proliferation and activation of fibroblasts. This leads to increase in type I collagen synthesis, overproduction of proinflammatory and pro-fibrotic cytokines, and worsens vascular Glutathione S transferases are a large superfamily that metabolize the detoxification of reactive-oxygen species of S-thiolated proteins that damage cells.…”

“…Decreased antioxidant capacity such as lower levels of serum alpha‐tocopherol, selenium, carotene and ascorbic acid, and higher values of oxidant molecules contribute to the oxidative stress mechanism in scleroderma pathogenesis 3 . In in‐vitro studies it has been shown that fibroblasts cultured from scleroderma lesions, release increased amounts of reactive oxidizing species which on the other hand induce proliferation and activation of fibroblasts.…”

“…It could be demonstrated that inhibition of ROS‐generating NADPH oxidase in vitro and in vivo, is capable to suppress the activated fibroblasts and experimental skin fibrosis 9,10 . Some of the oxidative stress neutralizing compounds such as anti‐oxidative enzymes including catalase, glutathione peroxidase, nonenzymatic compounds with antioxidant capacity such as vitamin C and E, selenium, glutathione, and oxidative stress markers such as lipid peroxidation products seem to contribute to fibrosis in morphea 3 …”

“…2 After it was reported that oxidative stress may cause scleroderma-like features, this mechanism has been also implicated in the pathogenesis of morphea. 3 Afterwards, an abnormal formation of reactive oxygen species (ROS) was shown in most of the pathologic features in systemic scleroderma patients. 4 Oxidative stress occurs as a result of the imbalance between prooxidant and antioxidant systems and causes cellular damage.…”

“…The etiology of morphea is still obscure and it is suggested that there is a genetic predisposition with an autoimmune basis in addition to environmental triggering factors 2 . After it was reported that oxidative stress may cause scleroderma‐like features, this mechanism has been also implicated in the pathogenesis of morphea 3 . Afterwards, an abnormal formation of reactive oxygen species (ROS) was shown in most of the pathologic features in systemic scleroderma patients 4 …”