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Yellow nail syndrome(YNS)

MedGen UID:
113164
Concept ID:
C0221348
Disease or Syndrome
Synonyms: LYMPHEDEMA AND YELLOW NAILS; Lymphedema with yellow nails; YNS
SNOMED CT: Hereditary lymphedema and yellow nails (400211001); Yellow nail syndrome (400211001)
 
Monarch Initiative: MONDO:0007921
OMIM®: 153300
Orphanet: ORPHA662

Definition

Yellow nail syndrome (YNS) is classically considered to comprise a clinical triad of yellow nails, lymphedema, and respiratory tract involvement. Two of these symptoms are required for the diagnosis, since the complete triad is only observed in about one-third of patients. Onset is usually after puberty (Hoque et al., 2007). [from OMIM]

Clinical features

From HPO
Hypoplasia of lymphatic vessels
MedGen UID:
871105
Concept ID:
C4025570
Congenital Abnormality
Congenital underdevelopment of lymph vessels.
Lymphedema
MedGen UID:
6155
Concept ID:
C0024236
Disease or Syndrome
Localized fluid retention and tissue swelling caused by a compromised lymphatic system.
Predominantly lower limb lymphedema
MedGen UID:
320552
Concept ID:
C1835228
Finding
Localized fluid retention and tissue swelling caused by a compromised lymphatic system, affecting mainly the legs.
Yellow nails
MedGen UID:
369197
Concept ID:
C1768507
Sign or Symptom
Yellowish discoloration of the nails.
Slow-growing nails
MedGen UID:
331935
Concept ID:
C1835238
Finding
Nails whose growth is slower than normal.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVYellow nail syndrome
Follow this link to review classifications for Yellow nail syndrome in Orphanet.

Professional guidelines

PubMed

Benassaia E, Abba S, Fourgeaud C, Mihoubi A, Vignes S
Dermatology 2024;240(2):343-351. Epub 2023 Dec 9 doi: 10.1159/000535577. PMID: 38071959
Li A, Sun Q
JAMA Dermatol 2022 Oct 1;158(10):1218-1219. doi: 10.1001/jamadermatol.2022.2533. PMID: 35947390
Makrilakis K, Pavlatos S, Giannikopoulos G, Toubanakis C, Katsilambros N
Ann Intern Med 2004 Aug 3;141(3):246-7. doi: 10.7326/0003-4819-141-3-200408030-00028. PMID: 15289232

Recent clinical studies

Etiology

Gutierrez CN, Low CM, Stokken JK, Choby G, O'Brien EK
Am J Rhinol Allergy 2020 Mar;34(2):156-161. Epub 2019 Oct 11 doi: 10.1177/1945892419881253. PMID: 31603343
Itkin M, McCormack FX
Clin Chest Med 2016 Sep;37(3):409-20. doi: 10.1016/j.ccm.2016.04.004. PMID: 27514588
Dhillon SS
Am J Respir Crit Care Med 2012 Sep 15;186(6):e10. doi: 10.1164/rccm.201109-1750IM. PMID: 22984032
Carnassale G, Margaritora S, Vita ML, Parisi AM, Congedo MT, Cusumano G, Granone PM
J Clin Oncol 2011 Mar 1;29(7):e156-8. Epub 2010 Dec 13 doi: 10.1200/JCO.2010.31.8402. PMID: 21149669
Nanda S, Dorville F
CMAJ 2009 Oct 27;181(9):614. Epub 2009 Sep 21 doi: 10.1503/cmaj.080255. PMID: 19770240Free PMC Article

Diagnosis

Kournoutas I, Tung EE
N Engl J Med 2024 Jul 4;391(1):68. doi: 10.1056/NEJMicm2401377. PMID: 38959483
Fustà-Novell X, Esquius-Rafat M
J Cutan Med Surg 2024 May-Jun;28(3):320. Epub 2024 Feb 5 doi: 10.1177/12034754241229366. PMID: 38314712
Braun CM, Ryu JH
Clin Chest Med 2021 Dec;42(4):667-675. doi: 10.1016/j.ccm.2021.08.003. PMID: 34774173
Sil A, Chandra A, Banik B, Biswas SK, Chakraborty U
J R Coll Physicians Edinb 2021 Jun;51(2):166-167. doi: 10.4997/JRCPE.2021.217. PMID: 34131678
Dhillon SS
Am J Respir Crit Care Med 2012 Sep 15;186(6):e10. doi: 10.1164/rccm.201109-1750IM. PMID: 22984032

Therapy

Li A, Sun Q
JAMA Dermatol 2022 Oct 1;158(10):1218-1219. doi: 10.1001/jamadermatol.2022.2533. PMID: 35947390
Omori T, Okamoto R, Fujimoto H, Masuda J, Kobayashi T, Fujii E, Takei Y, Ito M
Circ J 2018 Sep 25;82(10):2678-2679. Epub 2018 Mar 13 doi: 10.1253/circj.CJ-18-0154. PMID: 29540629
Dhillon SS
Am J Respir Crit Care Med 2012 Sep 15;186(6):e10. doi: 10.1164/rccm.201109-1750IM. PMID: 22984032
Maldonado F, Ryu JH
Curr Opin Pulm Med 2009 Jul;15(4):371-5. doi: 10.1097/MCP.0b013e32832ad45a. PMID: 19373089
Nelson LM
Arch Dermatol 1969 Oct;100(4):499-500. doi: 10.1001/archderm.100.4.499. PMID: 5358121

Prognosis

Cohen BA, Turcios NL
Pediatr Clin North Am 2021 Feb;68(1):261-276. doi: 10.1016/j.pcl.2020.09.009. PMID: 33228937
Ali MS, Ghori UK, Musani AI
Med Clin North Am 2019 May;103(3):503-515. doi: 10.1016/j.mcna.2018.12.009. PMID: 30955517
Dhillon SS
Am J Respir Crit Care Med 2012 Sep 15;186(6):e10. doi: 10.1164/rccm.201109-1750IM. PMID: 22984032
Nanda S, Dorville F
CMAJ 2009 Oct 27;181(9):614. Epub 2009 Sep 21 doi: 10.1503/cmaj.080255. PMID: 19770240Free PMC Article
Maldonado F, Ryu JH
Curr Opin Pulm Med 2009 Jul;15(4):371-5. doi: 10.1097/MCP.0b013e32832ad45a. PMID: 19373089

Clinical prediction guides

Abid N, Loukil M, Mahmoud N, Badri I, Bouzaidi K, Ghrairi H
Tunis Med 2021 Avril;99(4):475-477. PMID: 35244933Free PMC Article
Vignes S, Baran R
Orphanet J Rare Dis 2017 Feb 27;12(1):42. doi: 10.1186/s13023-017-0594-4. PMID: 28241848Free PMC Article
Woodfield G, Nisbet M, Jacob J, Mok W, Loebinger MR, Hansell DM, Wells AU, Wilson R
Respirology 2017 Jan;22(1):101-107. Epub 2016 Aug 23 doi: 10.1111/resp.12866. PMID: 27551950
DeCoste SD, Imber MJ, Baden HP
J Am Acad Dermatol 1990 Apr;22(4):608-11. doi: 10.1016/0190-9622(90)70081-r. PMID: 2319021
Norton L
Cutis 1985 Dec;36(6):457-62. PMID: 4075838

Recent systematic reviews

Valdés L, Huggins JT, Gude F, Ferreiro L, Alvarez-Dobaño JM, Golpe A, Toubes ME, González-Barcala FJ, José ES, Sahn SA
Respirology 2014 Oct;19(7):985-92. Epub 2014 Aug 14 doi: 10.1111/resp.12357. PMID: 25123563

Supplemental Content

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