Linear scleroderma

Description

scleroderma: Scleroderma is a rare autoimmune connective tissue disorder characterized by abnormal hardening of the skin and, sometimes, other organs. It is classified into two main forms: localized scleroderma and systemic sclerosis (SSc), the latter comprising three subsets; diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc) and limited SSc (lSSc) (see these terms).

Data source
FinnGen phenocode L12_LINEARSCLERODERMA
Hospital Discharge registry ICD-10: L941
Cause of Death registry ICD-10: L941
Level in the ICD-hierarchy 4
First defined in version DF2
Ontology
DOID 419
GWAS catalog 1001993
MESH D012594

Key figures

Sex All Female Male
Number of individuals 15 9 6
Unadjusted prevalence (%) 0.01 0.01 0.01
Mean age at first event (years) 40.2 37.24 44.63
Case fatality at 5-years (%) 0.0 0.0 0.0

Longitudinal metrics

Sex All Female Male
Median nb. of events per indiv. 2 1 3
Recurrence at 6 months (%) 46.67 33.33 66.67

Associations

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