Orphanet: Diffuse cutaneous systemic sclerosis

Search for a rare disease

* (*) mandatory field

Other search option(s)

Suggest an update

(*) Required fields.


Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed.

Captcha image

Diffuse cutaneous systemic sclerosis

Disease definition

Diffuse cutaneous systemic sclerosis (dcSSc) is a subtype of Systemic Sclerosis (SSc; see this term) characterized by truncal and acral skin fibrosis with an early and significant incidence of diffuse involvement (interstitial lung disease, oliguric renal failure, diffuse gastrointestinal disease, and myocardial involvement).


Classification level: Subtype of disorder
  • Synonym(s):
    • Diffuse cutaneous systemic scleroderma
    • Progressive cutaneous systemic scleroderma
    • Progressive cutaneous systemic sclerosis
  • Prevalence: -
  • Inheritance: Multigenic/multifactorial or Not applicable 
  • Age of onset: Adult
  • ICD-10: M34.0
  • OMIM: -
  • UMLS: -
  • MeSH: -
  • GARD: 9751
  • MedDRA: -

Detailed information


The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.