Twenty-nail dystrophy L60.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

20-nail dystrophy; dystrophy twenty nail dystrophy; Nail disorder, nonsyndromic congenital 1; NDNC1; OMIM 161050; sandpapered nails; Trachyonychia; Twenty-nail-dystrophy; Twenty Nail Syndrome

History
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Alkiewicz, 1950

Definition
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Clinically defined, non-syndromal, hereditary clinical picture with dystrophy of all (this definition is not tenable because not always 20 nails are changed) finger- and toenails (NDNC1). Here the nails are characterized by a rough, dull, sandpaper-like surface(trachyonychia). Other nail symptoms can also characterize this clinical picture.

S.a. Twenty-nail dystrophy of childhood

Occurrence/Epidemiology
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w>m

Manifestation
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In larger studies (n= 88) 2 manifestation peaks could be identified:

  • Childhood
  • Adults

Histology
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Spongiosis of the nail matrix and the nail bed, in some places vent shaped parakeratosis of the nails.

Therapy
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There is no known causal therapy for hereditary twenty-nail dystrophy.

In trachyonychia which occur in the context of underlying diseases, the treatment of the underlying disease is in the foreground; possibly short-term glucocorticoids under occlusion (e.g. glucocorticoid tinctures such as betamethasone in alcoholic-aqueous solution).

In case of resistance to therapy, the use of Ciclosporin A may be an alternative.

Otherwise, increased care of the nail plate.

Note(s)
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The clinically descriptive term "trachyonychia" is also defined differently in different publications. It is probably a pathological expression pattern of the nail organ with very different trigger mechanisms.

Trachonyonychia can also occur reactively in various underlying diseases (e.g. psoriasis vulgaris, atopic eczema, lichen planus, alopecia areata).

Literature
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  1. Alkiewicz J (1950) Trachyonychia. Ann Dermatol Venereol 10: 136-140
  2. Braun-Falco O et al (1981) Trachyonychia: 20-nail dystrophy. Dermatologist 32: 17-22
  3. Chien P Jr et al (2008) Alopecia universalis with twenty-nail dystrophy (trachyonychia). Dermatol Online J 14:24
  4. Commens, C. A. Twenty-nail dystrophy in identical twins. Pediatrics. Derm. 5: 117-119, 1988
  5. Grover C et al (2003) Longitudinal nail biopsy: utility in 20-nail dystrophy. Dermatol Surg 29: 1125-1129
  6. Khan S et al (2015) Genetics of human isolated hereditary nail disorders. Br J Dermatol 173:922-929.

  7. Lee YB et al (2012) Cyclosporine administration improves clinical manifestations and quality of life in patients with 20-nail dystrophy: case series and survey study. J Dermatol 39:1064-1065
  8. Wilson NJ et al (2013) Recessive mutations in the gene encoding frizzled 6 cause twenty nail dystrophy--expanding the differential diagnosis for pachyonychia congenita. J Dermatol Sci 70: 58-60

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Last updated on: 29.10.2020