Rare diseases · Sign or symptom
Preauricular skin tag
HP:0000384
What it means
A rudimentary tag of skin often containing ear tissue including a core of cartilage and located just anterior to the auricle (outer part of the ear).
Rare diseases that can present with this54
Very common80–99%
11- 14q22q23microdeletion syndrome
- Axial mesodermal dysplasia spectrum
- Branchiogenic deafness syndrome
- Cat-eye syndrome
- Craniofacial microsomia
- Hardikar syndrome
- Intellectual disability-hypoplastic corpus callosum-preauricular tag syndrome
- Mandibulofacial dysostosis-microcephaly syndrome
- Oculocerebrocutaneous syndrome
- Townes-Brocks syndrome
- Trisomy 18p syndrome
Common30–79%
18- Auriculocondylar syndrome
- BOR syndrome
- Emanuel syndrome
- Frontofacionasal dysplasia
- Frontonasal dysplasia-severe microphthalmia-severe facial clefting syndrome
- Frontorhiny
- Isotretinoin-like syndrome
- Lambert syndrome
- Lowe-Kohn-Cohen syndrome
- Macrostomia-preauricular tags-external ophthalmoplegia syndrome
- Microcephaly-deafness-intellectual disability syndrome
- Oculoauriculofrontonasal syndrome
- Oculoauriculovertebral spectrum with radial defects
- Oculocerebrofacial syndrome, Kaufman type
- Peters plus syndrome
- Severe microbrachycephaly-intellectual disability-athetoid cerebral palsy syndrome
- Trisomy 13 syndrome
- Trisomy 8p syndrome
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: Ear tag · Periauricular skin tag · Preauricular acrochordon · Preauricular fibroepithelial polyp · Preauricular skin tags · Preauricular tag · Preauricular tags · Skin tag in front of the ear
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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.