| What is Scleroderma? (MAIN MENU) |
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Types of Scleroderma: Overview |
| This page was written by Shelley Ensz and has not yet been medically edited. See disclaimer. |
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| Overview |
| There are many methods used to categorize the various forms of scleroderma, which has brought about a confusing abundance of terms. The following categories are often found in patient literature, but use and interpretation of these categories varies quite a bit. |
| Two Main Types |
There are two main types of scleroderma: Localized and Systemic. |
| The systemic forms can affect any part of the body (skin, blood vessels, and internal organs.) The systemic forms are also referred to as "systemic sclerosis" plus other terms such as diffuse, limited, CREST, and overlap. |
| The localized forms are morphea and linear. They affect only the skin (and sometimes the underlying tissues) but do not affect the internal organs, or reduce one's life expectancy in any way. |
| When any form of scleroderma (either localized or systemic) occurs in children, it is also called Childhood Scleroderma or Juvenile Scleroderma. |
| New scleroderma subtypes provide disease insight. One in five patients with clinical symptoms characteristic of systemic sclerosis does not fit into the popular classification scheme that divides the disease into the diffuse and limited. Undifferentiated scleroderma was defined as Raynaud's phenomenon and at least one additional major feature of systemic sclerosis in patients not meeting the full American College of Rheumatology criteria; such features include nail-fold capillary changes, pulmonary hypertension, puffy fingers, or scleroderma-specific autoantibodies. Jancin, Bruce. Entrpreneur. September 2008. (Also see: Undifferentiated and Overlap) |
| Determining the Type of Scleroderma |
| Profile of Pre-Scleroderma Evolution. All those patients who evolved to SSc presented the following at baseline: Raynaud's, positive anti-Scl-70 or anticentromere, sclerodactyly or visceral (esophageal) involvement and slow capillaroscopic pattern of scleroderma. E. Delgado. (THU0323) EULAR 2009. |
| Dartmouth researchers discover gene signatures for scleroderma. Distinct genetic profiles can discern different groups of patients with scleroderma. This discovery of distinguishing molecular subtypes within the disease offers new insight into the complexity of a poorly understood and hard to treat illness and opens a window for better diagnosis and targeted therapies. Genetic Engineering & Biotechnology News. July 15, 2008. (Also see: Causes of Scleroderma ) |
| The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. In this nationwide register, a descriptive classification of patients with disease manifestations characteristic of SSc in five groups allows to include a broader spectrum of patients with features of SSc. Rheumatology 2008 47(8):1185-1192. |
| Systemic sclerosis - diagnosis and classification. Early diagnosis and classification may be difficult if disease expression is oligosymptomatic (undifferentiated), presenting with only Raynaud's phenomenon or limited scleroderma. PubMed. Z Rheumatol. 2006 Jun 28. (Also see: Difficult Diagnosis) |
| Studies of scleroderma at The Alfred Hospital, Melbourne. All types have a high incidence of autoantibodies, but these are generally not related to the severity of the disease and do not occur in relatives or spouses. PubMed. Intern Med J. 2006 Aug;36(8):513-8. (Also see: Antibodies ) |
| Juvenile Scleroderma |
| Juvenile Scleroderma. When any form of scleroderma (either localized or systemic) occurs in children, it is also called Childhood Scleroderma or Juvenile Scleroderma. ISN. |
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| Localized |
| Localized Scleroderma.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. ISN. |
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| Nodular Systemic Scleroderma |
| Nodular Scleroderma: Case Report and Literature Review. Nodular Systemic Sclerosis is a rare variant that presents with lesions that clinically resemble keloids. Most patients had symptoms of systemic sclerosis. J Rheumatol. Volume 30: No. 11 November 2003;30:2500-2. (Also see: Diseases Similar to Scleroderma) |
| Systemic Sclerosis (Systemic Scleroderma) |
| Systemic scleroderma is also known as systemic sclerosis (SSc). There are several types, Limited/CREST and Diffuse. These types can affect the entire body, including the skin, blood vessels, and/or internal organs. ISN. |
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| Systemic Sclerosis: Prognosis and Mortality |
| Systemic Sclerosis (Scleroderma): Prognosis and Mortality. Now that the full range of types and severity of scleroderma are being recognized and diagnosed, there is increasing awareness that many people have a milder type of illness with a much rosier longterm prognosis. ISN. |
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| See Also |
| Juvenile Scleroderma by ISN. |
| Scleroderma Patient and Caregiver Stories by ISN. |
