Fibrosing connective tissue disorders of the skin: Molecular similarities and distinctions

The cytokines of pulmonary fibrosis: Much learned, much more to learn

“…The process of abnormal, structure-deforming, and function-impairing deposition of ECM is commonly referred to as fibrosis. It may occur in all organs and complicate a remarkably broad spectrum of diseases; its consequences are unpleasant and debilitating in the skin [1,2], and may be life-threatening in the liver [3][4][5], kidney [6,7], heart [8,9], or lung [10][11][12][13][14][15][16][17][18]. In both normal tissue healing and fibrosis, cells known as myofibroblasts are the main source of ECM accumulation, whether physiologic or pathologic [19,20].…”

“…Despite the frequent occurrence of organ fibrosis, the exact epidemiologic data on fibrosis in general are not readily available, likely due to the broad diversity of conditions in which fibrosis occurs as well as the common underreporting of fibrotic complications of diseases. It is obvious, however, that fibrosis of organs poses major biomedical problems, leading to notable contributions to morbidity and mortality [1][2][3][4][5][6][7][8][9][10][11][12]. The burden of organ fibrosis is further increased by the lack of sufficiently effective therapies.…”

Protease Activated Receptor-1 Deficiency Diminishes Bleomycin-Induced Skin Fibrosis

“…Injury of the skin can ultimately lead to scar formation, like keloid and hypertrophic scars (3). Although the clinical appearance of the different skin disorders might differ, the activation, proliferation and migration of resident fibroblasts at the site of trauma which induces deposition of extracellular matrix (ECM) proteins like fibronectin and collagen is a common pathway in these disorders (3)(4)(5). Targeting fibroblast activation and/or function may thus provide new therapeutic strategies in the treatment of fibroproliferative skin disorders due to its direct antifibrotic effect (4).…”

Attempts to accelerate wound healing

“…Within this phase, fibroplasia and angiogenesis take place concurrently in a closely orchestrated manner to form ECM and granulation tissue. Factors involved in fibroplasia have been intensively studied in systemic sclerosis, keloids, and their rodent models [14][15][16][17]. Scleroderma is characterized by excess production and deposition of ECM proteins, at least partly because fibroblasts in scleroderma are resistant to Fas-mediated apoptosis and prone to survive.…”