U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Congenital onychodystrophy

MedGen UID:
853249
Concept ID:
C1393669
Congenital Abnormality
Synonym: Congenital malformed nails
 
HPO: HP:0008394

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital onychodystrophy

Conditions with this feature

Ectodermal dysplasia 4, hair/nail type
MedGen UID:
870434
Concept ID:
C4024880
Disease or Syndrome
Some ectodermal dysplasias are here classified as congenital disorders characterized by abnormal development in 2 or more ectodermal structures (hair, nails, teeth, and sweat glands) without other systemic findings. Ectodermal dysplasia of the hair/nail type is a rare congenital condition characterized by hypotrichosis and nail dystrophy without nonectodermal or other ectodermal manifestations.

Professional guidelines

PubMed

Campeau PM, Hennekam RC; DOORS syndrome collaborative group
Am J Med Genet C Semin Med Genet 2014 Sep;166C(3):327-32. Epub 2014 Aug 28 doi: 10.1002/ajmg.c.31412. PMID: 25169651

Recent clinical studies

Etiology

Tona R, Inagaki S, Ishibashi Y, Faridi R, Yousaf R, Roux I, Wilson E, Fenollar-Ferrer C, Chien WW, Belyantseva IA, Friedman TB
J Biol Chem 2024 Sep;300(9):107725. Epub 2024 Aug 28 doi: 10.1016/j.jbc.2024.107725. PMID: 39214300Free PMC Article
Gao X, Dai P, Yuan YY
Hum Genet 2022 Apr;141(3-4):821-838. Epub 2021 Jul 7 doi: 10.1007/s00439-021-02310-2. PMID: 34232384
Campeau PM, Hennekam RC; DOORS syndrome collaborative group
Am J Med Genet C Semin Med Genet 2014 Sep;166C(3):327-32. Epub 2014 Aug 28 doi: 10.1002/ajmg.c.31412. PMID: 25169651
Poerink JG, Kon M, van Minnen LP
J Plast Reconstr Aesthet Surg 2011 Jun;64(6):822-6. Epub 2010 Oct 6 doi: 10.1016/j.bjps.2010.08.043. PMID: 20926359
Gobello T, Mazzanti C, Zambruno G, Chinni LM, Happle R
Dermatology 2000;201(1):51-3. doi: 10.1159/000018431. PMID: 10971062

Diagnosis

Bernal Masferrer L, Matei MC, Gilaberte Calzada Y, Navarro Campoamor L
Actas Dermosifiliogr 2024 Sep;115(8):T773-T780. Epub 2024 Jul 5 doi: 10.1016/j.ad.2024.07.011. PMID: 38972583
Lim SYD, Cheng WNS
Am Fam Physician 2023 Feb;107(2):118-119. PMID: 36791457
Gao X, Dai P, Yuan YY
Hum Genet 2022 Apr;141(3-4):821-838. Epub 2021 Jul 7 doi: 10.1007/s00439-021-02310-2. PMID: 34232384
Brennan CB, Buehler T, Lesher JL Jr
Pediatr Dermatol 2013 Jul-Aug;30(4):e52-3. Epub 2012 Feb 13 doi: 10.1111/j.1525-1470.2011.01668.x. PMID: 22329539
Qazi Q, Kassner EG
J Med Genet 1988 Aug;25(8):505-20. doi: 10.1136/jmg.25.8.505. PMID: 3050097Free PMC Article

Therapy

Armina S, Heiko T, Rudolf H, Judith F, Dimitra K, Peter S
J Dtsch Dermatol Ges 2020 Sep;18(9):1054-1057. Epub 2020 Jun 8 doi: 10.1111/ddg.14141. PMID: 32515052
Joy Way Bueno SM, Santos Muñoz A, Maldonado I, Larralde M
Pediatr Dermatol 2020 Jan;37(1):159-161. Epub 2019 Oct 20 doi: 10.1111/pde.14021. PMID: 31630427
Ali ME, Sikdar AU, Akhtar N, Islam ZM
Mymensingh Med J 2007 Jan;16(1):100-3. doi: 10.3329/mmj.v16i1.262. PMID: 17344790

Prognosis

Lozano R, Herman K, Rothfuss M, Rieger H, Bayrak-Toydemir P, Aprile D, Fruscione F, Zara F, Fassio A
Am J Med Genet A 2016 Dec;170(12):3207-3214. Epub 2016 Aug 19 doi: 10.1002/ajmg.a.37933. PMID: 27541164
Alessandri JL, Cuillier F, Malan V, Brayer C, Grondard M, Jacquemot-Dekkak L, Kieffer-Traversier M, Pierre F, Laurain C, Samperiz S, Tiran-Rajaofera I, Ramful D
Am J Med Genet A 2014 Mar;164A(3):648-54. Epub 2013 Dec 19 doi: 10.1002/ajmg.a.36323. PMID: 24357154
Poerink JG, Kon M, van Minnen LP
J Plast Reconstr Aesthet Surg 2011 Jun;64(6):822-6. Epub 2010 Oct 6 doi: 10.1016/j.bjps.2010.08.043. PMID: 20926359

Clinical prediction guides

Arif T, Amin SS, Adil M, Mohtashim M
Acta Dermatovenerol Croat 2017 Jul;25(2):161-163. PMID: 28871934
Lozano R, Herman K, Rothfuss M, Rieger H, Bayrak-Toydemir P, Aprile D, Fruscione F, Zara F, Fassio A
Am J Med Genet A 2016 Dec;170(12):3207-3214. Epub 2016 Aug 19 doi: 10.1002/ajmg.a.37933. PMID: 27541164
Rasool M, Nawaz S, Azhar A, Wajid M, Westermark P, Baig SM, Klar J, Dahl N
Eur J Dermatol 2010 Jul-Aug;20(4):443-6. Epub 2010 Apr 21 doi: 10.1684/ejd.2010.0962. PMID: 20409997
Gobello T, Mazzanti C, Zambruno G, Chinni LM, Happle R
Dermatology 2000;201(1):51-3. doi: 10.1159/000018431. PMID: 10971062
Perry HO, Su WP
Semin Dermatol 1995 Jun;14(2):145-51. doi: 10.1016/s1085-5629(05)80011-2. PMID: 7640195

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...