%0 Journal Article
%T Updates on Morphea: Role of Vascular Injury and Advances in Treatment
%A Julio C. Sartori-Valinotti
%A Megha M. Tollefson
%A Ann M. Reed
%J Autoimmune Diseases
%D 2013
%I Hindawi Publishing Corporation
%R 10.1155/2013/467808
%X Morphea and systemic sclerosis are fibrosing disorders of the skin that share common inflammatory and immunologic pathways that are responsible for the vascular changes, increased collagen production, and extracellular matrix proliferation seen in both conditions. Recent advances in molecular biology techniques have furthered our knowledge of the potential underlying pathogenic mechanisms and offer new and provocative areas of research for novel diagnostic and therapeutic interventions. This review focuses on the role of vascular injury in the development of morphea, the use of ultrasonography as a diagnostic modality, and well-established and newly proposed treatments. 1. Introduction Morphea is an inflammatory, fibrosing skin disorder that leads to sclerosis of the dermis and subcutaneous tissue but in some cases may also extend to the fascia, muscle, and underlying bone. Clinically, morphea has an asymmetric distribution and is usually confined to one body area; hence it is also referred to as localized scleroderma. Systemic sclerosis (SSc), however, in addition to symmetric skin changes is characterized by internal organ involvement, sclerodactyly, presence of Raynaud¡¯s phenomenon, and nailfold capillary abnormalities. Despite these differences, both entities share common inflammatory and immunologic pathways that are ultimately responsible for the vascular changes, increased collagen production, and extracellular matrix proliferation seen in both conditions. Although the etiology and precise mechanisms that trigger the cascade of molecular events that culminate in skin fibrosis are not fully understood, advances in molecular biology techniques have furthered our knowledge of the potential culprits and offer new and provocative areas of research for novel diagnostic and therapeutic interventions. This brief review will focus on the role of vascular injury in the development of morphea with emphasis on recent basic research data as well as use of ultrasonography as a diagnostic method. Lastly, well-established and newly proposed treatments will be discussed. 2. Clinical Features Several classification systems have been developed in attempt to grasp the breath of the various forms of presentation of morphea [1, 2]. They are largely based on clinical findings and include, with minor differences, at least four major variants: plaque-type, linear, generalized and a miscellaneous group of morphologically distinct phenotypes. Plaque-Type Morphea (Morphea en Plaque or Circumscribed). It is the most common subtype overall and the most common variant in
%U http://www.hindawi.com/journals/ad/2013/467808/