Are You Confident of the Diagnosis?
What you should be alert for in the history
Since trachyonychia can be associated with a variety of dermatologic disorders, clinical history should include questions about prior dermatoses, including lichen planus, psoriasis, and alopecia areata. The patient may have been treated with antifungal medications in the past without successful resolution of the nail changes.
Characteristic findings on physical examination
Trachyonychia refers to rough nails, and there are two clinical variants. The first type is called opaque trachyonychia. It features a nail plate that demonstrates longitudinal ridges; the nails are opaque, rough, and have a “sandpapered” appearance.
The second type is called shiny trachyonychia. It features a nail plate that contains numerous small pits, arranged in both longitudinal and parallel lines. The nails have a shiny appearance, and the nail plate is not opaque (Figure 1).
Expected results of diagnostic studies
Generally, trachyonychia can be diagnosed on clinical appearance alone, but it is reasonable to perform a nail clipping for histology to evaluate for the presence of fungus as part of an evaluation of onychodystrophy.
In the evaluation of trachyonychia, nail unit biopsies most often show a spongiotic pattern in the matrix. If the trachyonychia is caused by lichen planus or psoriasis, corresponding histopathologic features from a nail matrix specimen can be identified.
It is important to examine all twenty nails, and to perform a complete skin exam. The presence of characteristic lesions of a particular dermatosis can help connect the nail changes of trachyonychia to that dermatosis.
Since cases of trachyonychia are treated similarly despite their cause, a nail unit biopsy is not required in order to proceed with therapy, if desired.
Idiopathic trachyonychia is defined as isolated nail involvement in the absence of a personal history of signs of dermatological disease.
The diagnosis of trachyonychia is most challenging when the nails alone are affected. Onychomycosis can sometimes have a similar clinical appearance, and performing a nail clipping for histology will evaluate for this possibility.
Other primary dermatoses affecting the nail unit such as psoriasis and lichen planus (not presenting as trachyonychia) could also be considered in the differential diagnosis.
Who is at Risk for Developing this Disease?
Trachyonychia has been associated with a variety of dermatoses; most commonly, alopecia areata, psoriasis, and lichen planus. Patients with known diagnoses of these disorders could be at risk.
The diagnosis of idiopathic trachyonychia has been reported in 1.5% (45 of 2951) of patients evaluated for nail disorders. In a separate study, trachyonychia was noted in 3.65% (40 of 1095) of alopecia areata patients.
What is the Cause of the Disease?
Most cases of trachyonychia that have been biopsied demonstrate a spongiotic dermatitis. Trachyonychia related to alopecia areata will show a spongiotic dermatitis within the nail matrix histologically. Trachyonychia, which is related to lichen planus or psoriasis, will show the respective histology in a nail matrix specimen.
Trachyonychia has also been associated with a variety of other conditions, including atopic dermatitis, graft-versus-host disease, ichthyosis vulgaris, immunoglobulin A deficiency, incontinentia pigmenti, koilonychia, pemphigus vulgaris, primary biliary cirrhosis, trauma, vitiligo, and reflex sympathetic dystrophy.
The nail changes seen in opaque trachyonychia are caused by inflammation that affects the entire nail matrix and moderates in intensity, but is continuous.
In shiny trachyonychia, inflammation periodically affects the nail matrix, but is interspersed with episodes of normal nail matrix functioning.
Systemic Implications and Complications
Idiopathic trachyonychia is not associated with systemic involvement.
Trachyonychia is a nonscarring process, and treatment is not mandatory. Many cases of trachyonychia will spontaneously resolve. Single cases or small case series can be found in the literature that report improvement with a variety of interventions, but none is favored.
For shiny trachyonychia, use of nail cosmetics as a camouflage mechanism is reasonable.
In these single and small case studies, local therapies which have been reported to improve trachyonychia include intralesional steroids. A variety of strengths have been reported, including (2.5 to 3mg/ml), and 10mg/ml. It is prudent to begin with a lower concentration to avoid possible local side effects.
Other local therapies include tazarotene 0.1% gel, topical psoralen and ultraviolet A (PUVA), topical steroids (personally I have had the most success with intralesional steroid injections in combination with tazarotene gel), and urea cream (Sakata et al. specifically note that in three of their patients with trachyonychia, a cuticle and nail cream that contained 6% urea was used).
Successful oral therapies include oral biotin and pulse dose oral steroids. Mittal et al. report treating a 12-year-old girl with trachyonychia affecting most of the fingernails and toenails with 4mg of betamethasone as a single oral dose with breakfast on 2 consecutive days every week and no treatment on the other 5 days of the week. In two months, there was clear improvement in the proximal one-third of the nails, and the nails were normal by 6 months.
Tosti et al. reported a patient with trachyonychia from nail lichen planus who was treated with oral prednisolone at a dose of 0.5mg/kg on alternate days for 4 weeks. Four months after stopping the therapy, the nails showed only mild longitudinal ridging. The patient did not have recurrence of the nail changes during a follow-up period of 3 years.
Biotin has reported to demonstrate improvement in trachyonychia when used at a dose of 2.5mg/day for 6 months, in two patients.
Psoriatic trachyonychia specifically has been successfully treated with 5% 5-fluorouracil (5-FU) cream and acitretin. Tosti et al. report successful treatment of psoriatic trachyonychia in a 38-year-old patient with acitretin at a daily dose of 0.3mg/kg. After 3 months, the nails were improved, and after 7 months, the nail changes had completely resolved.
Schissel et al. report treating a 48-year-old patient with psoriatic trachyonychia with 5% topical 5-FU cream every other day for 20 minutes and then washed off. After 2 weeks of therapy, the treatment was changed to an every fourth day application because of irritation. Improvement was seen over 16 weeks of treatment, and the patient was maintained on a once-weekly 20-minute application of the medication.
Optimal Therapeutic Approach for this Disease
The reported therapies above are from single case reports or very small case series, so an evidence-based approach does not favor a particular therapeutic maneuver.
As trachyonychia is a nonscarring process, and many cases will spontaneously improve, no treatment or observation is a preferred plan. If the patient desires treatment, camouflage with nail cosmetics for shiny trachyonychia is a good option and is well tolerated. I have seen improvement in some cases with intralesional steroids.
Use of urea and tazarotene also have a favorable side effect profile, when used for trachyonychia. Most patients do not want to proceed to oral medications for this condition.
Nails take months to see appreciable changes in response to therapy, so it is important to advise patients to allow at least 3 months to assess whether a particular regimen has had an effect.
Trachyonychia itself does not require monitoring. If the patient is undergoing treatment, it is reasonable to assess the response after a few months of medication use.
Unusual Clinical Scenarios to Consider in Patient Management
Aside from being most commonly associated with lichen planus or psoriasis, trachyonychia has also been associated with a variety of other conditions, including atopic dermatitis, graft-versus-host disease, ichthyosis vulgaris, immunoglobulin A deficiency, incontinentia pigmenti, koilonychia, pemphigus vulgaris, primary biliary cirrhosis, trauma, vitiligo, and reflex sympathetic dystrophy.
What is the Evidence?
Sakata, S, Howard, A, Tosti, A, Sinclair, R. “Follow up of 12 patients with trachyonychia”. Australasian Journal of Dermatology. vol. 47. 2006. pp. 166-8. (Twelve patients with trachyonychia were treated with therapies, including various delivery methods of corticosteroids (non-oral) and urea cream. Total resolution or marked improvement was seen in 50% of the patients after an average period of 42 months, regardless of the treatment.)
Tosti, A, Fanti, P, Morelli, R, Bardazzi, F. “Trachyonychia associated with alopecia areata: a clinical and pathologic study”. J Am Acad Dermatol. vol. 25. 1991. pp. 266-70. (Twelve patients with alopecia areata and nail changes that fulfilled clinical criteria for trachyonychia had nail unit biopsies performed. Spongiotic changes were noted in eleven of these patients. The final patient showed pathologic changes of lichen planus, and this patient developed lichen planus of the skin 6 months after the nail unit biopsy.
Overall, of 1095 patients with alopecia areata, 40 had trachyonychia, comprising 3.65% of the population, and trachyonychia was interpreted to be an uncommon nail manifestation of alopecia areata.)
Tosti, A, Piraccini, B, Iorizzo, M. “Trachyonychia and related disorders: evaluation and treatment plans”. Dermatologic Therapy. vol. 15. 2002. pp. 121-5. (A comprehensive review of trachyonychia, its associations, and reported therapies)
Blanco, F, Scher, R. “Trachyonychia: Case report and review of the literature”. J Drugs in Dermatol. vol. 5. 2006. pp. 469-72. (This article describes a 45-year-old woman who developed trachyonychia associated with systemic sarcoidosis. A matrix biopsy demonstrated spongiotic trachonychia. This article contains a comprehensive review of trachyonychia and its associations.)
Tosti, A, Bardazzi, F, Piraccini, B, Fanti, P. “Idiopathic trachyonychia (twenty-nail dystrophy): a pathological study of 23 patients”. Br J Dermatol. vol. 131. 1994. pp. 866-72. (Idiopathic trachyonychia refers to trachyonychia of the nails without a personal history or signs of dermatologic disease. Twenty-three patients with idiopathic trachyonychia who had nail unit biopsies were studied. Histologically, nineteen patients had spongiotic changes, three patients showed psoriasiform changes, and one patient showed lichen planus.)
Soda, R, Diluvio, L, Bianchi, L, Chimenti, S. “Treatment of trachyonychia with tazarotene”. Clinical and Experimental Dermatology. vol. 30. 2005. pp. 294-307. (A 36-year-old man with alopecia universalis developed severe trachyonychia of all twenty nails. Significant improvement was noted with a regimen of tazarotene 0.1% gel applied once daily overnight on the affected nail plates, surrounding nail folds, and periungual skin for 3 months. Side effects were minimal. A second course of this regimen cleared the trachyonychia after a relapse.)
Mittal, R, Khaitan, B, Sirka, C. “Trachyonychia treated with oral mini pulse therapy”. Indian Journal of Dermatology, Venereology and Leprology. vol. 67. 2001. pp. 202-3. (A 12-year-old girl with trachyonychia was treated successfully with oral betamethasone.)
Tosti, A, Bellavista, S, Iorizzo, M, Vincenzi, C. “Occupational trachyonychia due to psoriasis: report of a case successfully treated with oral acitretin”. Contact Dermatitis. vol. 54. 2006. pp. 123-4. (A 38-year-old man was successfully treated with acitretin at 0.3mg/kg for psoriatic trachyonychia.)
Schissel, D, Elston, D. “Topical 5-fluorouracil treatment for psoriatic trachyonychia”. Cutis. vol. 62. 1998. pp. 27-8. (A 48-year-old woman with psoriatic trachyonychia improved with treatment with topical 5-fluorouracil.)
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