Rare diseases · Sign or symptom

Perioral hyperpigmentation

Darkening of skin around the mouth

HP:0010802

What it means

Increased pigmentation, either focal or generalized, of the skin surrounding the vermilion of the lips.

Periorbital hyperpigmentation may be accompanied by Lip freckles, but this should be assessed separately.

Rare diseases that can present with this1

Common30–79%

1

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: Increased pigmentation around the mouth

Perioral hyperpigmentation

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This 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.