Rare diseases · Sign or symptom
Melanocytic nevus
Beauty mark
HP:0000995
What it means
A oval and round, colored (usually medium-to dark brown, reddish brown, or flesh colored) lesion. Typically, a melanocytic nevus is less than 6 mm in diameter, but may be much smaller or larger.
Melanocytic nevi are commonly known as moles. The majority of moles appear during the first 20 to 30 years of a person's life, however, some may be present when the baby is born. The average human has from 10 to 14 moles. Moles can develop on the scalp, under the nails, armpits, virtually anywhere on the body. The total number of moles a person can have usually varies during his or her lifetime.
Rare diseases that can present with this39
Very common80–99%
13- ADULT syndrome
- Arterial dissection-lentiginosis syndrome
- Cutis gyrata-acanthosis nigricans-craniosynostosis syndrome
- Distal deletion 17q syndrome
- Gastrocutaneous syndrome
- Gorlin syndrome
- Hypo- and hypermelanotic cutaneous macules-retarded growth-intellectual disability syndrome
- Linear nevus sebaceus syndrome
- Neurocutaneous melanocytosis
- Neurofibromatosis type 1
- Noonan syndrome with multiple lentigines
- Proteus syndrome
- X-linked intellectual disability-cubitus valgus-dysmorphism syndrome
Common30–79%
10- 14q11.2microdeletion syndrome
- Beckwith-Wiedemann syndrome
- Camptodactyly syndrome, Guadalajara type 1
- Congenital amegakaryocytic thrombocytopenia
- Cowden syndrome
- Dermoodontodysplasia
- Ectodermal dysplasia, trichoodontoonychial type
- Facial dysmorphism-anorexia-cachexia-eye and skin anomalies syndrome
- Hypohidrotic ectodermal dysplasia-hypothyroidism-ciliary dyskinesia syndrome
- Oculocutaneous albinism type 1B
Sometimes5–29%
16- 15q13.3microdeletion syndrome
- Acrodysostosis
- Alopecia-epilepsy-pyorrhea-intellectual disability syndrome
- Congenital infiltrating lipomatosis of the face
- Crouzon syndrome
- Hermansky-Pudlak syndrome
- Hypotrichosis with juvenile macular degeneration
- Juvenile Paget disease
and 8 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, and Orphanet separately records 1 disease where this sign is specifically absent. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Melanocytic naevus · Melanocytic nevi · Nevocellular nevi · Noncancerous mole · Pigmented naevi · Pigmented nevi
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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.