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Classification and epidemiology of scleroderma
1Division of Rheumatology, Wayne State University, Detroit, MI 48201, USA.
Abstract:
Scleroderma is classified as two separate but related entities, a localized form and a systemic form. The classification scheme for morphea presented here is that of Peterson et al, which divides morphea into five categories: plaque, generalized, bullous, linear, and deep. Using this system, these authors estimated the incidence rate of localized scleroderma to be 27 new cases per million population per year. Overall survival was similar to that of the general population. There was a preponderance of female cases (approximately 3:1) for all forms of morphea except for linear scleroderma, which had an even sex distribution. Systemic scleroderma is divided into limited and diffuse disease based on the extent of skin involvement. Recent estimates have placed the incidence rate of systemic sclerosis in the United States at 19 new cases per million adults per year, with an overall prevalence of 240/million adults. The female-to-male ratio is approximately 5:1. The prevalence of scleroderma varies by geographic region and ethnic background and is higher in the United States than in Europe or Japan. Although systemic sclerosis survival has improved over the past two decades, with 5-year survival over 80%, long-term survival is significantly lower than expected, and morbidity is considerable.
Insights
Scleroderma, a condition affecting skin and connective tissues, has distinct localized and systemic forms. While localized scleroderma has a similar survival rate to the general population, systemic sclerosis, though improving, still presents considerable long-term morbidity.
Area of Science:
- Dermatology
- Rheumatology
- Epidemiology
Background:
- Scleroderma encompasses localized (morphea) and systemic (scleroderma) forms.
- Morphea classification includes plaque, generalized, bullous, linear, and deep types.
- Systemic sclerosis is categorized into limited and diffuse disease based on skin extent.
Purpose of the Study:
- To outline the classification and epidemiology of localized and systemic scleroderma.
- To present incidence and prevalence rates for different scleroderma subtypes.
- To discuss survival and morbidity associated with scleroderma.
Main Methods:
- Utilized the Peterson et al. classification system for morphea.
- Reviewed incidence and prevalence data for localized scleroderma and systemic sclerosis.
- Analyzed demographic data, including sex distribution and geographic/ethnic variations.
Main Results:
- Localized scleroderma incidence: 27 new cases/million/year; predominantly affects females (3:1), except linear type.
- Systemic sclerosis incidence: 19 new cases/million adults/year; prevalence: 240/million adults; female predominance (5:1).
- Scleroderma prevalence varies geographically and ethnically; higher in the US than Europe/Japan.
Conclusions:
- Scleroderma classification aids in understanding distinct disease entities.
- While systemic sclerosis survival has improved, long-term morbidity remains significant.
- Epidemiological data highlights variations in scleroderma occurrence and demographics.