NDNC1
MCID: NLD009
MIFTS: 39

Nail Disorder, Nonsyndromic Congenital, 1 (NDNC1)

Categories: Bone diseases, Fetal diseases, Genetic diseases, Rare diseases, Skin diseases

Aliases & Classifications for Nail Disorder, Nonsyndromic Congenital, 1

MalaCards integrated aliases for Nail Disorder, Nonsyndromic Congenital, 1:

Name: Nail Disorder, Nonsyndromic Congenital, 1 58
Twenty-Nail Dystrophy 58 12 54
Nonsyndromic Congenital Nail Disorder 1 12 15
Idiopathic Trachyonychia 54 60
Twenty Nail Dystrophy 54 74
Nail Disorder, Nonsyndromic Congenital 1 54
Onychodystrophy Totalis, Isolated 58
Autosomal Dominant Nail Dysplasia 54
Onychodystrophy Totalis 54
Sandpaper Nails 54
Trachyonychia 54
Ndnc1 58

Characteristics:

Orphanet epidemiological data:

60
idiopathic trachyonychia
Inheritance: Autosomal dominant; Age of onset: Childhood; Age of death: normal life expectancy;

OMIM:

58
Inheritance:
autosomal dominant

Miscellaneous:
no abnormalities of skin, hair, teeth, or bones
not all nails are affected in some patients


HPO:

33
nail disorder, nonsyndromic congenital, 1:
Onset and clinical course slow progression
Inheritance autosomal dominant inheritance


Classifications:

Orphanet: 60  
Rare skin diseases


External Ids:

Disease Ontology 12 DOID:0080079
OMIM 58 161050
ICD10 via Orphanet 35 L60.3
UMLS via Orphanet 75 C0406443
Orphanet 60 ORPHA79153
MedGen 43 C4551561
UMLS 74 C0406443

Summaries for Nail Disorder, Nonsyndromic Congenital, 1

OMIM : 58 Many types of nonsyndromic congenital nail disorders (NDNC) have been described. Twenty-nail dystrophy (TND), also known as trachyonychia (from the Greek for 'rough nails'), is an autosomal dominant nail dystrophy characterized by excessive longitudinal striations and numerous superficial pits on the nails, which have a distinctive rough sandpaper-like appearance. Occasionally some nails are spared. The slowly progressive condition is usually apparent at birth and may be self-limiting, with spontaneous resolution in some patients (summary by Sehgal, 2007). TND is referred to here as nonsyndromic congenital nail disorder-1 (NCNC1). (161050)

MalaCards based summary : Nail Disorder, Nonsyndromic Congenital, 1, also known as twenty-nail dystrophy, is related to alopecia and nail disorder, nonsyndromic congenital, 6. An important gene associated with Nail Disorder, Nonsyndromic Congenital, 1 is FZD6 (Frizzled Class Receptor 6). The drugs Wormwood and Mugwort have been mentioned in the context of this disorder. Affiliated tissues include skin and bone, and related phenotypes are thick nail and onycholysis

NIH Rare Diseases : 54 Twenty-nail dystrophy is a condition that affects the nails of the fingers and toes. The nails become rough, thin, and brittle. They appear similar to sandpaper. It was once thought that all 20 nails will be affected, but any number of nails can become rough and brittle. This condition most commonly occurs in childhood, but it can occur at any age. The exact cause is often unknown, but sometimes appears with other skin conditions such as lichen planus, psoriasis and alopecia areata. In some people, the symptoms of twenty-nail dystrophy improve or disappear over time, but in other people they persist for a long time.

Related Diseases for Nail Disorder, Nonsyndromic Congenital, 1

Diseases in the Nail Disease family:

Nail Disorder, Nonsyndromic Congenital, 6 Nail Disorder, Nonsyndromic Congenital, 2
Nail Disorder, Nonsyndromic Congenital, 3 Nail Disorder, Nonsyndromic Congenital, 1
Nail Disorder, Nonsyndromic Congenital, 5 Nail Disorder, Nonsyndromic Congenital, 4
Nail Disorder, Nonsyndromic Congenital, 7 Nail Disorder, Nonsyndromic Congenital, 8
Nail Disorder, Nonsyndromic Congenital, 9 Nail Disorder, Nonsyndromic Congenital, 10

Diseases related to Nail Disorder, Nonsyndromic Congenital, 1 via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 31)
# Related Disease Score Top Affiliating Genes
1 alopecia 29.7 TP63 AIRE
2 nail disorder, nonsyndromic congenital, 6 11.0
3 nail disorder, nonsyndromic congenital, 2 11.0
4 nail disorder, nonsyndromic congenital, 3 11.0
5 nail disorder, nonsyndromic congenital, 5 11.0
6 nail disorder, nonsyndromic congenital, 4 11.0
7 nail disorder, nonsyndromic congenital, 7 11.0
8 nail disorder, nonsyndromic congenital, 8 11.0
9 nail disorder, nonsyndromic congenital, 9 11.0
10 nail disorder, nonsyndromic congenital, 10 11.0
11 lichen planus 10.4
12 alopecia universalis congenita 10.3
13 ichthyosis vulgaris 10.2
14 ichthyosis 10.2
15 alopecia areata 10.2
16 cutis laxa 10.1
17 darier-white disease 10.0
18 pachyonychia congenita 1 10.0
19 incontinentia pigmenti 10.0
20 immunoglobulin alpha deficiency 10.0
21 down syndrome 9.9
22 reflex sympathetic dystrophy 9.9
23 childhood type dermatomyositis 9.9
24 dermatomyositis 9.9
25 algoneurodystrophy 9.9
26 turner syndrome 9.9
27 lichen nitidus 9.9
28 psoriasis 9.9
29 amyloidosis 9.9
30 acquired cutis laxa 9.9
31 nail disease 9.6 FZD6 AIRE

Graphical network of the top 20 diseases related to Nail Disorder, Nonsyndromic Congenital, 1:



Diseases related to Nail Disorder, Nonsyndromic Congenital, 1

Symptoms & Phenotypes for Nail Disorder, Nonsyndromic Congenital, 1

Human phenotypes related to Nail Disorder, Nonsyndromic Congenital, 1:

33
# Description HPO Frequency HPO Source Accession
1 thick nail 33 occasional (7.5%) HP:0001805
2 onycholysis 33 HP:0001806
3 thin nail 33 HP:0001816
4 trachyonychia 33 HP:0030804

Symptoms via clinical synopsis from OMIM:

58
Skin Nails Hair Nails:
slowly progressive
distal onycholysis
rough, sandpaper-like fingernails and toenails
longitudinal ridging, excessive (trachyonychia)
superficial pitting (in some patients)
more

Clinical features from OMIM:

161050

MGI Mouse Phenotypes related to Nail Disorder, Nonsyndromic Congenital, 1:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 no phenotypic analysis MP:0003012 9.13 AIRE PROS1 TP63
2 normal MP:0002873 8.92 AIRE FZD6 PROS1 TP63

Drugs & Therapeutics for Nail Disorder, Nonsyndromic Congenital, 1

Drugs for Nail Disorder, Nonsyndromic Congenital, 1 (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Wormwood Approved, Experimental Phase 3
2 Mugwort Phase 3

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Pilot Study of Efficacy of Nail Gel in Finger Nail Surface Abnormality Unknown status NCT02582762 Phase 3 Nail gel

Search NIH Clinical Center for Nail Disorder, Nonsyndromic Congenital, 1

Genetic Tests for Nail Disorder, Nonsyndromic Congenital, 1

Anatomical Context for Nail Disorder, Nonsyndromic Congenital, 1

MalaCards organs/tissues related to Nail Disorder, Nonsyndromic Congenital, 1:

42
Skin, Bone

Publications for Nail Disorder, Nonsyndromic Congenital, 1

Articles related to Nail Disorder, Nonsyndromic Congenital, 1:

(show all 47)
# Title Authors Year
1
Twenty-Nail Dystrophy: Revisited. ( 29911530 )
2018
2
Rare Presentation of Alopecia Universalis Congenita and Twenty-nail Dystrophy in Siblings. ( 28839389 )
2017
3
Therapeutic Efficacy of Combination Therapy Using Oral Cyclosporine with a Dietary Supplement (Pantogar®) in Twenty-Nail Dystrophy. ( 28966518 )
2017
4
Trachyonychia and Twenty-Nail Dystrophy: A Comprehensive Review and Discussion of Diagnostic Accuracy. ( 27843915 )
2016
5
Twenty-Nail Dystrophy and Darier's (Darier-White) Disease. ( 26861433 )
2015
6
Recessive mutations in the gene encoding frizzled 6 cause twenty nail dystrophy--expanding the differential diagnosis for pachyonychia congenita. ( 23374899 )
2013
7
Clinical study of twenty-nail dystrophy in Korea. ( 21913910 )
2012
8
Twenty nail dystrophy in association with zosteriform lichen planus. ( 22837581 )
2012
9
Segmental vitiligo and twenty-nail dystrophy: an unusual association. ( 19172003 )
2008
10
Alopecia universalis with twenty-nail dystrophy (trachyonychia). ( 18627760 )
2008
11
Twenty nail dystrophy trachyonychia: an overview. ( 17535400 )
2007
12
Twenty nail dystrophy in vitiligo. ( 17291300 )
2007
13
Twenty-nail dystrophy originating from lichen planus. ( 15654171 )
2005
14
Twenty-nail dystrophy (trachyonychia) caused by lichen planus in a patient with gold allergy. ( 15888190 )
2005
15
Twenty-nail dystrophy in a girl with incontinentia pigmenti. ( 12581087 )
2003
16
Twenty-nail dystrophy in a mother and her 7-year-old daughter associated with balanced translocation 46, XX, t(6q13;10p13). ( 12072794 )
2002
17
An association of twenty-nail dystrophy with vitiligo. ( 11280463 )
2001
18
Twenty-nail dystrophy in monozygotic twins. ( 10343962 )
1999
19
Twenty-nail dystrophy and vitiligo: a rare association. ( 10071326 )
1999
20
A case report of twenty-nail dystrophy. ( 9046745 )
1997
21
Twenty-nail dystrophy (trachyonychia) caused by lichen planus in a patient with alopecia universalis and ichthyosis vulgaris. ( 7593807 )
1995
22
Idiopathic trachyonychia (twenty-nail dystrophy): a pathological study of 23 patients. ( 7857841 )
1994
23
Twenty-nail dystrophy due to lichen planus in a patient with alopecia areata. ( 8348737 )
1993
24
Twenty-nail dystrophy associated with hematologic abnormalities. ( 1755311 )
1991
25
Twenty-nail dystrophy of childhood with koilonychia. ( 2007303 )
1991
26
Twenty-nail dystrophy treated with topical PUVA. ( 1981426 )
1990
27
Twenty-nail dystrophy. A clinical manifestation of spongiotic inflammation of the nail matrix. ( 2383032 )
1990
28
Twenty nail dystrophy in identical twins. ( 3412992 )
1988
29
Twenty Nail Dystrophy with Pterygium Unguis. ( 28134145 )
1988
30
Twenty-nail dystrophy of childhood: a misnamed syndrome. ( 3608573 )
1987
31
Twenty-nail dystrophy of childhood: a reappraisal. ( 3881224 )
1985
32
Twenty-nail dystrophy: report of a case and review of the literature. ( 6203314 )
1984
33
Twenty-nail dystrophy of childhood: a sign of localized lichen planus. ( 6494064 )
1984
34
Twenty-nail dystrophy: a hypothesis. ( 6703749 )
1984
35
Twenty-nail dystrophy of childhood--two cases in one family. ( 6499277 )
1984
36
IgA in twenty-nail dystrophy. ( 6886924 )
1983
37
Familial severe twenty-nail dystrophy. ( 7130495 )
1982
38
Twenty-nail dystrophy (trachyonychia) associated with selective IgA deficiency. ( 7062176 )
1982
39
Hereditary twenty-nail dystrophy in a Sicilian family. ( 7143386 )
1982
40
Twenty-nail dystrophy and ichthyosis vulgaris. ( 7247418 )
1981
41
Twenty-nail dystrophy of alopecia areata. ( 7377792 )
1980
42
Twenty-nail dystrophy of childhood. Case report and histopathological findings. ( 427021 )
1979
43
Twenty-nail dystrophy. ( 434859 )
1979
44
[Vertical striated sand-papered twenty-nail dystrophy (author's transl)]. ( 686612 )
1978
45
Twenty-nail dystrophy: a variant of lichen planus. ( 646381 )
1978
46
Twenty-nail dystrophy of childhood. ( 831628 )
1977
47
Twenty-nail dystrophy. ( 911187 )
1977

Variations for Nail Disorder, Nonsyndromic Congenital, 1

Expression for Nail Disorder, Nonsyndromic Congenital, 1

Search GEO for disease gene expression data for Nail Disorder, Nonsyndromic Congenital, 1.

Pathways for Nail Disorder, Nonsyndromic Congenital, 1

GO Terms for Nail Disorder, Nonsyndromic Congenital, 1

Biological processes related to Nail Disorder, Nonsyndromic Congenital, 1 according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 hair follicle development GO:0001942 8.62 FZD6 TP63

Molecular functions related to Nail Disorder, Nonsyndromic Congenital, 1 according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 transcription regulatory region DNA binding GO:0044212 8.62 AIRE TP63

Sources for Nail Disorder, Nonsyndromic Congenital, 1

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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