Diffuse cutaneous systemic sclerosis
ORPHA:220393· ICD-10 M34.0
Definition
Diffuse cutaneous systemic sclerosis (dcSSc) is a subtype of Systemic Sclerosis (SSc) 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).
- Prevalence
- 1-9 / 100 000
- Inheritance
- Multigenic/multifactorial, Not applicable
- Age of onset
- Adult