Rare diseases · Sign or symptom
Tented upper lip vermilion
HP:0010804
What it means
Triangular appearance of the oral aperture with the apex in the midpoint of the upper vermilion and the lower vermilion forming the base.
This finding is distinguished from an Exaggerated Cupid's bow by the alteration of the shape of the oral aperture.
Rare diseases that can present with this40
Very common80–99%
10- Congenital-onset Steinert myotonic dystrophy
- Dysmorphism-pectus carinatum-joint laxity syndrome
- FOXG1 syndrome due to 14q12 microdeletion
- Fryns syndrome
- Kleefstra syndrome
- Progressive spondyloepimetaphyseal dysplasia-short stature-short fourth metatarsals-intellectual disability syndrome
- Proximal Xq28 duplication syndrome
- Smith-Magenis syndrome
- Waardenburg syndrome type 3
- X-linked intellectual disability, Cantagrel type
Common30–79%
16- 20q11.2microduplication syndrome
- 2q23.1microdeletion syndrome
- Autosomal recessive Robinow syndrome
- Basel-Vanagaite-Smirin-Yosef syndrome
- Birk-Barel syndrome
- Hyperphosphatasia-intellectual disability syndrome
- Hypotonia-speech impairment-severe cognitive delay syndrome
- Keipert syndrome
- Keppen-Lubinsky syndrome
- Microcephaly-facial dysmorphism-ocular anomalies-multiple congenital anomalies syndrome
- Microform holoprosencephaly
- PLAA-associated neurodevelopmental disorder
- Severe neonatal hypotonia-seizures-encephalopathy syndrome due to 5q31.3 microdeletion
- Waardenburg syndrome type 1
- X-linked alpha-thalassemia-intellectual disability syndrome
- X-linked intellectual disability, Stevenson type
Sometimes5–29%
14- 20p13microdeletion syndrome
- Congenital multicore myopathy with external ophthalmoplegia
- DPM1-CDG
- Fumaric aciduria
- Intellectual disability-brachydactyly-Pierre Robin syndrome
- Intellectual disability-seizures-hypophosphatasia-ophthalmic-skeletal anomalies syndrome
- Kagami-Ogata syndrome due to paternal uniparental disomy of chromosome 14
- Multiple congenital anomalies-hypotonia-seizures syndrome
and 6 more in this range
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Inverted V-shaped upper lip · Tented mouth · Tented upper lip
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.