Presented by: Jaryd Freedman, MD, Catherine Warner, MD, and Mark Abdelmalek, MD
Drexel University College of Medicine
May 16, 2016
Twenty-nail dystrophy is a rare condition in which all nails simultaneously become dystrophic. Most commonly the nail plate will show trachyonychia; however, other dystrophic features may be present. The condition is usually a manifestation of a primary cutaneous disease but is often idiopathic. Twenty-nail dystrophy is most often associated with alopecia areata, lichen planus, psoriasis, or vitiligo, though many other dermatoses have been reported concomitantly.
The differential diagnosis includes common conditions such as eczema, chronic paronychia, trauma and fungal infections. However, it would be rare for all twenty-nails to become involved simultaneously in these conditions.
Twenty-nail dystrophy is typically an acquired sporadic condition, although there have been several family cohorts described who have a non-syndromic inherited form of twenty-nail dystrophy, transmitted in an autosomal dominant fashion. No associated gene mutation has been discovered to date. In addition, many syndromic genodermatoses may present with dystrophy of all twenty-nails. These include but are not limited to, pachyonychia congenita, nail patella syndrome, dyskeratosis congenita, ectodermal dysplasias along with inherited immunodeficiencies.
The clinical findings of twenty-nail dystrophy are due to inflammation involving the nail matrix. Cases in which the etiology of the twenty-nail dystrophy is unclear may be differentiated by a longitudinal matrix biopsy. However, as this risks causing permanent nail dystrophy and will be unlikely to influence treatment, most authors do not advise nail biopsies as standard protocol in these patients.
There is no well-established treatment for twenty-nail dystrophy. In many patients the disorder will be self-limited, while others will take on a chronic persistent course. Topical, intralesional and systemic corticosteroids, topical and oral retinoids, PUVA, and cyclosporine have all been tried with mixed results. Generally twenty-nail dystrophy is not associated with nail unit scarring even when associated with lichen planus. A conservative approach to treatment may be advisable.
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