KEGG   DISEASE: Yellow nail syndrome
Entry
H03009                      Disease                                
Name
Yellow nail syndrome
Description
Yellow nail syndrome (YNS) is a rare disorder characterized by a triad of nail abnormalities, chronic respiratory disease, and primary lymphoedema. Because most patients lack a positive family history, and given the typically late onset of the disease as well as the potential recovery of nail changes, YNS is generally not considered to be a primarily genetic disease. However, recent studies have suggested that defects in planar cell polarity (PCP) organization may play an important role in the pathogenesis of YNS.
Category
Skin disease
Brite
Human diseases in ICD-11 classification [BR:br08403]
 14 Diseases of the skin
  Skin disorders involving specific cutaneous structures
   Disorders of the epidermis or epidermal appendages
    Disorders of the nail or perionychium
     EE11  Acquired abnormalities of nail colour
      H03009  Yellow nail syndrome
Pathway-based classification of diseases [BR:br08402]
 Signal transduction
  nt06505  WNT signaling
   H03009  Yellow nail syndrome
 Cellular processes
  nt06549  Cadherin signaling
   H03009  Yellow nail syndrome
Pathway
hsa04519 Cadherin signaling   
Network
nt06505 WNT signaling
nt06549 Cadherin signaling
Gene
CELSR1 [HSA:9620] [KO:K04600]
Other DBs
ICD-11: EE11.1
MeSH: D056684
OMIM: 153300
Reference
  Authors
Kurolap A, Chai Gadot C, Eshach Adiv O, Hershkovitz T, Avitan-Hersh E, Martin L, Humeau H, Schatz UA, Westphal DS, Lobmaier S, Sofrin-Drucker E, Stafler P, Bugis J, Chermesh I, Hardak E, Geva P, Zohar Y, Hershkovitz D, Mory A, Chatterji S, Greenberger S, Shteinberg M, Baris Feldman H
  Title
Impaired Wnt/Planar Cell Polarity Signaling in Yellow Nail Syndrome.
  Journal
Ann Intern Med 178:39-49 (2025)
DOI:10.7326/ANNALS-24-01101
Reference
  Authors
Hoque SR, Mansour S, Mortimer PS
  Title
Yellow nail syndrome: not a genetic disorder? Eleven new cases and a review of the literature.
  Journal
Br J Dermatol 156:1230-4 (2007)
DOI:10.1111/j.1365-2133.2007.07894.x
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