Nail Disorder, Nonsyndromic Congenital, 1 (NDNC1)

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OMIM®: ⁵⁷ 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 (NDNC1). (161050) (Updated 08-Dec-2022)

MalaCards based summary: Nail Disorder, Nonsyndromic Congenital, 1, also known as nonsyndromic congenital nail disorder 1, is related to nail disease and ameloonychohypohidrotic syndrome, and has symptoms including nail bed hemorrhage and onychomadesis. An important gene associated with Nail Disorder, Nonsyndromic Congenital, 1 is FZD6 (Frizzled Class Receptor 6). The drugs Miconazole and Clotrimazole have been mentioned in the context of this disorder. Affiliated tissues include skin, breast and lung, and related phenotypes are nail dystrophy and fingernail dysplasia

Orphanet ⁵⁸ Autosomal recessive nail dysplasia: Autosomal recessive nail dysplasia is a rare, isolated nail anomaly characterized by claw-shaped, thick, hyperplastic, hard and hyperpigmented nails, subungual hyperkeratosis, onycholysis and slow nail growth. Variable degree of disease severity has been reported.

Idiopathic trachyonychia: A rare isolated nail anomaly characterized by brittle, thin, rough, opaque appearing nails with excessive longitudinal ridging. In a less severe form, the nails retain their luster and present with superficial ridging and multiple small geometric pits. In both varieties, superficial scaling of the nail plate and hyperkeratosis of the cuticles, as well as koilonychia and onychoschizia are observed. Any number of nails may be affected, and fingernails are more often affected than toenails. Spontaneous improvement of the condition may occur.

MedlinePlus Genetics: ⁴² Nonsyndromic congenital nail disorder 10 is a condition that affects the fingernails and toenails. Affected individuals have extremely thick nails (onychauxis) that separate from the underlying nail bed (onycholysis) and can appear claw-like. Some fingers and toes may be missing part of the nail (hyponychia).In affected individuals, the nails are often abnormal from birth. However, the abnormalities may not be noticeable until later in childhood because the nails tend to grow more slowly than normal.Individuals with nonsyndromic congenital nail disorder 10 do not have any other health problems related to the condition.

GARD: ¹⁹ 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.

UniProtKB/Swiss-Prot: ⁷³ An autosomal recessive nail disorder characterized by a variable degree of onychauxis (thick nails), hyponychia, and onycholysis of all nails, with claw-shaped fingernails in some individuals. No other anomalies of ectodermal tissues, including hair, teeth, sweat glands, or skin, are noted, and individuals with dysplastic nails have normal hearing and normal psychomotor development.

Disease Ontology: ¹¹ A nonsyndromic congenital nail disorder that is characterized by excessive longitudinal striations and numerous superficial pits on the nails, which have a distinctive rough, sand paper-like appearance.

Clinical features from OMIM®:

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