raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:662

Lymphedema with yellow nails

Synonyms: YNS · Yellow nail syndrome

Type
Disease
Group
Disorder
Age of onset
Adult, Childhood, Neonatal
Status
active

A rare, syndromic nail anomaly disease characterized by the variable triad of characteristic yellow nails, chronic respiratory manifestations, and primary lymphedema.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enLymphedema with yellow nailsYNS · Yellow nail syndrome
frLymphoedème avec ongles jaunesSyndrome des ongles jaunes · YNS
deLymphödem mit gelben NägelnGelbe-Nägel-Syndrom · YNS
esLinfedema y uñas amarillasSíndrome de las uñas amarillas
itLinfedema con unghie gialleSindrome delle unghie gialle · Linfedema con unghie gialle · YNS
nlLymfoedeem met gele nagelsGelenagelsyndroom · YNS
ptSíndrome das unhas amarelas
plZespół żółtych paznokciObrzęk limfatyczny z żółtymi paznokciami · YNS
csSyndrom žlutých nehtůLymfedém se žlutými nehty · YNS
ukСиндром жовтих нігтівЛімфедема з жовтими нігтями · YNS
zh黄指甲综合征淋巴水肿伴黄指甲
trSarı tırnak sendromuYNS · Sarı tırnak ile seyreden lenfödem

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
OMIM153300Evalidated
ICD-10L60.5NTBTattributedvalidated
ICD-11EE11.1Especificvalidated
MONDO0007921Evalidated
MeSHD056684Evalidated
UMLSC0221348Evalidated
GARD184Evalidated
MedDRA10048244Evalidated

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 (3):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

2 records
TypeClassValueAreaQualificationValidationSource
Cases/families400 casesWorldwideCase(s)Validated28241848[PMID]
Point prevalence<1 / 1 000 000—WorldwideClass onlyValidatedORPHANET

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

24 annotations · validated 2016-06-01
HPOTerm (as annotated by Orphanet)Diagnostic
Very frequent (99-80%)
HP:0001231Abnormal fingernail morphology
HP:0008388Abnormal toenail morphology
HP:0002110Bronchiectasis
HP:0100798Fingernail dysplasia
HP:0003759Hypoplasia of lymphatic vessels
HP:0001004Lymphedema
HP:0100797Toenail dysplasia
HP:0011367Yellow nails
Frequent (79-30%)
HP:0012735Cough
HP:0002094Dyspnea
HP:0002102Pleuritis
HP:0002205Recurrent respiratory infections
HP:0012384Rhinitis
HP:0000246Sinusitis
Occasional (29-5%)
HP:0100574Biliary tract neoplasm
HP:0011354Generalized abnormality of skin
HP:0002721Immunodeficiency
HP:0002664Neoplasm
HP:0100526Neoplasm of the lung
HP:0000112Nephropathy
HP:0001806Onycholysis
HP:0002092Pulmonary arterial hypertension
HP:0009726Renal neoplasm
HP:0100242Sarcoma

Run the differential on the 8 obligate / very frequent terms

7

Sources for this card