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Classification and epidemiology of scleroderma

M D Mayes1

  • 1Division of Rheumatology, Wayne State University, Detroit, MI 48201, USA.

Seminars in Cutaneous Medicine and Surgery
|March 25, 1998
PubMed
Summary

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.

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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.

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  • 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.