uncombable hair syndrome
MONDO:0008621Mondo
Findings
No curated finding names uncombable hair syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Uncombable hair syndrome (UHS), or pili trianguli et canaliculi, is a rare scalp hair shaft dysplasia.
Definition from the Mondo Disease Ontology (MONDO:0008621), read 2026-09-29. CC BY 4.0.
Features
6 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abnormal hair morphologyHPOHP:0001595
- Very frequent (80% to 99% of cases)
- Coarse hairHPOHP:0002208
- Very frequent (80% to 99% of cases)
- TrichodysplasiaHPOHP:0002552
- Very frequent (80% to 99% of cases)
- White hairHPOHP:0011364
- Very frequent (80% to 99% of cases)
- Woolly hairHPOHP:0002224
- Very frequent (80% to 99% of cases)
- Patchy alopeciaHPOHP:0002232
- Occasional (5% to 29% of cases)
Genes
2 genes
Germline variants in the gene encoding each protein, as classified by GenCC submitters, each in their own words. Limited, disputed and refuted submissions are listed too.
- HGNC:18337HGNC:18337
- Supportive · Orphanet · Autosomal recessive · 2021
- TGM3HGNC:11779
- Supportive · Orphanet · Autosomal recessive · 2021
Where it sits
- A kind of
Other names
1 name
Resolves to: uncombable hair syndrome
- Also called
- pili trianguli et canaliculi