| Types of Scleroderma: Overview |
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| Localized Scleroderma |
| Morphea, Linear, En Coup de Sabre, Parry-Rombergs |
| This page was written by Shelley Ensz and has not yet been medically edited. See disclaimer. |
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| Overview |
| There are two types of localized scleroderma: Linear and Morphea. Localized scleroderma affects the skin. It may also affect the underlying muscles and bones, but it does not affect internal organs, and it does not reduce a person's life expectancy. (Also see: What is Scleroderma? and Types of Scleroderma) |
| In general, localized scleroderma is relatively mild, and may be related to systemic scleroderma only in terms of similar superficial symptoms, such as the appearance of skin biopsy under the microscope. |
| Localized Fibrosing Disorders: Linear Scleroderma, Morphea, Regional Fibrosis. Localized fibrosing disorders include several clinical and histopathological conditions that are similar to the skin involvement of systemic sclerosis, but the systemic features are absent. Localized fibrosing disorders can be classified into several subtypes that include morphea, generalized morphea, and linear scleroderma, in which facial involvement is termed en coup de sabre. eMedicine.com. 11/07/06. (Also see: Morphea) |
| Localized Scleroderma There is a wide spectrum of localized scleroderma, with types ranging from mild to disabling. eMedicine Dermatology. |
| Localized Scleroderma: Linear |
| Linear scleroderma is a line of thickened skin which can affect the bones and muscles underneath it, thus limiting the motion of the affected joints and muscles. It most often occurs in the arms, legs, or forehead, and may occur in more than one area. ISN. |
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| Localized Scleroderma: Morphea |
| Morphea scleroderma usually begins as patches of yellowish or ivory-colored rigid, dry skin. These then become hard, slightly depressed, oval-shaped plaques which usually have a whitish or yellowish center surrounded by a pinkish or purplish halo. ISN. |
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| Localized Scleroderma: Linear/Morphea |
| Linear morphea scleroderma. Sometimes "morphea" is used as a generic term for localized scleroderma of any type. Thus, sometimes the term "linear morphea" refers to localized scleroderma of any type (either linear or morphea). |
| When "linear morphea" is used more literally, it means that there is a mixture of both types -- linear and morphea, occurring together in combination. |
| Extracutaneous Manifestations (Symptoms beyond skin involvement) |
| Muscle Cramps Associated with Localized Scleroderma Skin Lesions: Focal Dystonia, Neuromyotonia, or Nerve Entrapment? Based on the temporal and spatial correlation of skin lesions and muscle cramps in our patients, we propose that localized scleroderma may precipitate muscle cramps, possibly caused by local nerve injury. J Rheumatol 2006 December;33:2549. Letter. |
| Chronic venous insufficiency (CVI) - a potential trigger for localized scleroderma. It may be that CVI is a potential trigger factor for LS, which may only develop if a certain amount of trigger factors are present and resolves if one or more of the contributing factors (such as CVI) can be treated. PubMed. J Eur Acad Dermatol Venereol. 2006 Jan;20(1):96-9. |
| Localized scleroderma in childhood is not just a skin disease. Extracutaneous manifestations of juvenile localized scleroderma developed in almost one-fourth of the children in this study. These extracutaneous manifestations often were unrelated to the site of the skin lesions and sometimes were associated with multiple organ involvement. The risk of developing SSc was very low. PubMed. Arthritis Rheum. 2005 Sep 2;52(9):2873-2881. |
| Localized scleroderma is an autoimmune disorder. Many previous studies conclude that localized scleroderma involves autoimmune abnormalities and is one of the organ-specific autoimmune disorders targeting mainly skin, although the types of autoimmune abnormality are different from systemic sclerosis. PubMed. Rheumatology (Oxford). 2004 Nov 23. (Also see: Morphea, Linear, and Dr. Shinichi Sato) |
| Collagen Degradation |
| Collagen Degradation Products And Elastin In Systemic (SSc) And Localized Scleroderma (lSSc) . Increased markers of collagen and elastin turnover in SSc and LSc reflect the active fibrotic process in the diseases and are accordance with the published data. High elastin levels in psoriasis vulgaris group are rather difficult to explain. R. Becvar FRI0245 EULAR 2008. (Also see: Systemic Scleroderma, and Skin Involvement ) |
| Collagen Degradation Products And Inflammatory Activity In Systemic (SSc) And Localized Scleroderma (LSc). In patients with SSc our data have shown the most intensive collagen degradation and simultanously an active inflammation which reflects the pathological processes in the skin and visceral organs, compared with psoriasis vulgaris patients and healthy inviduals. In LSc group collagen degradation was similar to that in control groups but a certain inflammatory activity was observed. R. Becvar THU0242 EULAR 2007. (Also see: Systemic Scleroderma and Skin Involvement ) |
| Patient and Caregiver Stories |
| Linear Stories |
| Morphea Stories |
| Juvenile Scleroderma by ISN. |
| Scleroderma Patient and Caregiver Stories by ISN. |
