Systemic and localized scleroderma.
Sclerosing conditions of the skin are manifested by a full spectrum of presentations that includes skin-limited forms as well as those which can involve internal organs and result in death. At this point, we are just beginning to understand the mechanisms of tissue fibrosis, and it is likely that the fibrotic processes are a heterogeneous group of disorders in which perturbation of multiple molecular pathways, including vascular and immunologically mediated pathways, can lead to fibrosis. We now have some moderately effective therapies for vascular aspects of systemic sclerosis (eg, bosentan for pulmonary arterial hypertension, calcium-channel blockers for Raynaud's, or angiotensin-converting enzyme inhibitors for renal crisis). We also are beginning to find treatments interrupting the immunologic pathways that manifest as systemic sclerosis (eg, methotrexate for the skin or cyclophosphamide for the lungs). The basic process of fibrosis, however, awaits proven, effective therapy.
Chung
Lorinda
L
Department of Dermatology, Stanford University School of Medicine, CA 94305, USA.
Lin
Jan
J
Furst
Daniel E
DE
Fiorentino
David
D
D000982:Antipruritics / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005355:Fibrosis; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D026741:Physical Therapy Modalities; D012594:Scleroderma, Localized / Q000175:diagnosis / Q000209:etiology / Q000473:pathology* / Q000628:therapy; D012595:Scleroderma, Systemic / Q000175:diagnosis / Q000209:etiology / Q000473:pathology* / Q000628:therapy; D012867:Skin / Q000473:pathology / Q000503:physiopathology; D012871:Skin Diseases / Q000175:diagnosis / Q000209:etiology / Q000473:pathology* / Q000628:therapy
D000982:Antipruritics; D007166:Immunosuppressive Agents