Thiel-Behnke corneal dystrophy
Findings
No curated finding names Thiel-Behnke corneal dystrophy yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
Thiel-Behnke corneal dystrophy (TBCD) is a rare form of superficial corneal dystrophy characterized by sub-epithelial honeycomb-shaped corneal opacities in the superficial cornea, and progressive visual impairment.
Definition from the Mondo Disease Ontology (MONDO:0011185), read 2026-09-29. CC BY 4.0.
Features
9 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.
- Central corneal dystrophyHPOHP:0007881
- Very frequent (80% to 99% of cases)
- Slow decrease in visual acuityHPOHP:0007924
- Very frequent (80% to 99% of cases)
- Subepithelial corneal opacitiesHPOHP:0008039
- Very frequent (80% to 99% of cases)
- Episodic painHPOHP:0032148
- Frequent (30% to 79% of cases)
- Ocular painHPOHP:0200026
- Frequent (30% to 79% of cases)
- Opacification of the corneal stromaHPOHP:0007759
- Frequent (30% to 79% of cases)
- PhotophobiaHPOHP:0000613
- Frequent (30% to 79% of cases)
- Recurrent corneal erosionsHPOHP:0000495
- Frequent (30% to 79% of cases)
- AstigmatismHPOHP:0000483
- Occasional (5% to 29% 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.
- TGFBIHGNC:11771
- Definitive · G2P · Autosomal dominant · 2017
- Supportive · Orphanet · Autosomal dominant · 2021
Where it sits
Other names
9 names
Resolves to: Thiel-Behnke corneal dystrophy
- Also called
- anterior limiting membrane dystrophy type 2anterior limiting membrane dystrophy type IIcorneal dystrophy of Bowman layer type 2corneal dystrophy of Bowman layer type IIcurly fiber corneal dystrophycurly fibre corneal dystrophyhoneycomb corneal dystrophyTBCDWaardenburg-Jonker corneal dystrophy