alopecia universalis congenita
Findings
No curated finding names alopecia universalis congenita yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
The most severe form of alopecia areata, an inflammatory disease of the hair follicle, which is characterized by a complete loss of hair of the scalp and all the hair-bearing areas of the body.
Definition from the Mondo Disease Ontology (MONDO:0008757), read 2026-09-29. CC BY 4.0.
- Inheritance
- Autosomal recessive inheritance
- Onset and course
- Neonatal onset
HPO, annotations 2026-09-02
Features
15 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.
- Absent axillary hairHPOHP:0002221
- 11 of 11 reported patients
- Absent eyebrowHPOHP:0002223
- 11 of 11 reported patients
- Very frequent (80% to 99% of cases)
- Absent eyelashesHPOHP:0000561
- 11 of 11 reported patients
- Very frequent (80% to 99% of cases)
- Absent pubic hairHPOHP:0002555
- 11 of 11 reported patients
- Alopecia universalisHPOHP:0002289
- 11 of 11 reported patients · Neonatal onset
- Very frequent (80% to 99% of cases)
- Patchy alopeciaHPOHP:0002232
- Very frequent (80% to 99% of cases)
Show the remaining 3
- Psoriasiform dermatitisHPOHP:0003765
- Very rare (1% to 4% of cases)
- Type I diabetes mellitusHPOHP:0100651
- Very rare (1% to 4% of cases)
- VitiligoHPOHP:0001045
- Very rare (1% to 4% of cases)
Genes
1 gene
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.
- HRHGNC:5172
- Strong · PanelApp Australia · Autosomal recessive · 2025
- Moderate · G2P · Autosomal recessive · 2025
- Supportive · Orphanet · Autosomal recessive · 2021
Where it sits
- A kind of
Other names
2 names
Resolves to: alopecia universalis congenita
- Also called
- atrichia, generalisedatrichia, generalized