raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:263432

Nevus of Ito

Synonyms: Nevus fuscocaeruleus acromiodeltoideus

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
All ages
Status
active

Nevus of Ito is a benign dermal melanocytosis occurring most frequently in the Asian populations and characterized by unilateral, asymptomatic, blue, gray or brown skin pigmentation within the acromioclavicular and upper chest area (involving the side of the neck, the supraclavicular and scapular areas, and the shoulder region). It is usually diagnosed in early infancy and in early adolescence. Nevus of Ito may progressively enlarge and darken in color (particularly with puberty) and its appearance usually remains stable once adulthood is reached. Spontaneous regression does not occur. Malignant melanoma has rarely been reported within a nevus of Ito. It shares the clinical features of nevus of Ota, except its anatomic location and in rare occasions, may occur together with the latter.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enNevus of ItoNevus fuscocaeruleus acromiodeltoideus
frNaevus d'ItoNaevus fuscocaeruleus acromiodeltoideus
deNaevus ItoNaevus fuscocaeruleus acromiodeltoides
esNevo de ItoNevo de Ota acromiodeltoide
itNevo di ItoNevo fuscoceruleo acromiodeltoideo
nlNaevus van ItoNaevus fuscocaeruleus acromiodeltoideus
ptNevus de Ito
plZnamię Ito= en
csItův névus= en
ukНевус ІтоНевус фускокаерулеус акроміодельтоідеус (Nevus fuscocaeruleus acromiodeltoideus)
zh伊藤痣肩峰三角肌蓝褐痣
trIto nevüsüNevüs fussuloserulös akromiodeltoides

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10D22.6NTBTattributedvalidated
ICD-11LC10NTBTindexvalidated
MONDO0016985Evalidated
UMLSC0022283Evalidated
GARD10830Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

2 records
TypeClassValueAreaQualificationValidationSource
Point prevalence1-9 / 100 0001.17 / 100 000EuropeValue and classNot yet validatedORPHANET
Point prevalenceUnknown—WorldwideClass onlyNot yet validatedORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

3 annotations · validated 2024-09-30
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0003764Nevus
Very rare (<4-1%)
HP:0012056Cutaneous melanoma
Excluded (0%)
HP:0025534Ocular melanocytosis

Run the differential on the 1 obligate / very frequent terms

Annotation source: 32809409[PMID]_26260725[PMID]

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Sources for this card