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Diseases of the Esophagus pp 383–393Cite as

Motor Disorders Due to Collagen Diseases

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Part of the book series: Handbuch der inneren Medizin ((INNEREN 5,volume 3))

Abstract

Progressive systemic sclerosis is a generalized disease of unknown origin, involving many organs (Goetz, 1945; Tuffanelli and Winkelmann, 1961). It produces hardness of the skin, fibrosis, loss of smooth muscle of internal organs and progressive loss of visceral and cutaneous functions (Winkelmann, 1971). Muscles, bones, heart, lungs, mucous membranes and the gastrointestinal tract may be involved. Several types of systemic sclerosis have been described: (1) Acrosclerosis is the more common form of systemic scleroderma (95%). It is characterized by Raynaud’s phenomenon, which is often the initial symptom. The disease usually begins on the hands and later spreads to other structures. The incidence in women is 2.6 to 2.9 times that in men (Leinwand et al., 1954; Tuffanelli and Winkelmann, 1961; D’Angelo et al., 1969; Poirier and Rankin, 1972). (2) Acute diffuse scleroderma, or malignant scleroderma, is a rare condition (5% of the patients with systemic sclerosis), characterized by generalized cutaneous sclerosis and usually beginning centrally. It has a rapid onset and course, and a poor prognosis. Raynaud’s phenomenon is absent. Malignant sclero derma affects as many men as women.

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  1. J. Hellemans
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Hellemans, J., Vantrappen, G. (1974). Motor Disorders Due to Collagen Diseases. In: Diseases of the Esophagus. Handbuch der inneren Medizin, vol 3. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-86429-2_19

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