[Scleroderma and fibrosing diseases]

Fibroblasts and myofibroblasts play an important role in the pathogenesis of systemic sclerosis, fibromatoses, arthrofibrosis, and Ormond's disease. These conditions are characterized by an excessive fibroblast proliferation and partly accompanied by inflammation. Scleroderma is either localized or systemic, and features additional vasculopathy. Scleroderma-like skin lesions can be found in graft-versus-host disease following allogeneic hematopoietic stem cell transplantation, complicated malignoma or can represent an adverse drug reaction. The fibromatoses are found in superficial, or as semi-malignant desmoids in deep body compartments. Ormond's disease is a chronic periaortitis of unknown origin which extends into the retroperitoneal space. The diagnostic relevance of a histopathological diagnosis of fibrosing diseases varies and ranges from a disease-supporting to a disease-defining value.