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Home , Alopecia areata, Nail disease

A Case of Twenty Dystrophy and Review of Treatment Options Emily Tongdee, BS,* Shahjahan Shareef, BS,** Tracy Favreau, DO,*** Khasha Touloei, DO****

*Medical student, Florida International University Herbert Wertheim College of , Miami, FL **Medical student, Nova Southeastern University College of Osteopathic Medicine, Ft. Lauderdale, FL ***Dermatologist, Nova Southeastern University College of Osteopathic Medicine, Ft. Lauderdale, FL **** resident, 3rd year, Nova Southeastern University College of Osteopathic Medicine, Ft. Lauderdale, FL

Disclosures: None Correspondence: Shahjahan Shareef; [email protected]

Abstract Twenty nail dystrophy (TND), also known as trachyonychia, is an abnormality of the proximal nail matrix. It presents as a homogenous roughness, giving the nail a sandpaper-like appearance.1- 3 Idiopathic trachyonychia most commonly presents with spongiotic with exocytosis of inflammatory cells.1,3-5 This disease has been shown to spontaneously resolve within about five to six years, but that may be too long for some patients, as this nail disorder can be cosmetically disfiguring, further impacting quality of life.6,7 We present a case of idiopathic TND and provide an updated review of the literature and the various treatments that have been utilized to treat the condition. Griseofulvin injections, PUVA, systemic steroids, oral , cyclosporine A, and nail plate dressings have shown to be highly successful treatment options. TND is a self-limiting disease. If treatment is sought, various options are available that can shorten the disease course, although no one particular treatment is considered the gold standard.

along the nail surface, longitudinal ridging and/ idiopathic twenty nail dystrophy, as all twenty Introduction 15 Twenty nail dystrophy (TND), also known as or pitting are almost always observed. In severe nails were involved and no associations were seen. trachyonychia, was first mentioned by Hazelrigg cases, TND may present with a sandpaper- We presented the patient with many different et al. in 1977 to describe nail dystrophy occurring like, opaque appearance. Severity of disease treatment options, including intralesional steroid in all 20 nails of six children.8 However, since not may vary between nails, but generally, the nails injections. Our patient elected to take 20 mg of evolve over time into a muddy, white-grayish biotin daily. She was also given reassurance. all 20 nails are always affected in this condition, 15,18 it has since been termed “trachyonychia.”1 discoloration. Trachyonychia is a specific type of proximal Discussion matrix abnormality that is marked by diffuse Case Report The etiology of TND is controversial. It can either homogenous roughness.2 In several case studies, A 34-year-old Filipino female presented to the present as its own entity or as a manifestation of it has been reported as an autosomal-dominant clinic with in all 20 of her nails, another condition, including (4% condition of idiopathic origin.9-12 TND has also starting with the thumb and progressing one by to 18.5% of cases), (45% to 83% been described in monozygotic twins. Girls and one beginning about two years earlier. She had of cases), or (13% to 26% of cases).1,19-22 boys are affected equally, and the condition can seen previous dermatologists who said fungal TND has also been associated with incontinentia occur in adults as well.13-16 Often, the disease cultures were negative and was empirically treated pigmenti, , vulgaris, ichthyosis, presents at birth and evolves slowly, or it may with oral for months with immunoglobulin IgA deficiency, hemolytic present in infancy or childhood and progress no improvement. She denied any significant abnormalities, koilonychias, eczema, primary from there.15,17 The peak age range is from 3 past medical history, surgical history, and biliary cirrhosis, and to 12 years old.3 It is more commonly seen in hospitalizations. Her family history was significant sarcoidosis.6,8,13-16,19,20,22-31 Alopecia areata is males than females when associated with alopecia for mellitus and hypertension in the most common disease associated with areata.3 her father and mother, respectively. She denied trachyonychia.2,18 Most commonly, TND presents as nail dystrophy any use of alcohol, tobacco or illicit and said Trachyonychia has a pathognomonic presentation with excessive longitudinal ridging and striations she was not taking any prescription medications, of thin, brittle nails with longitudinal ridging that give the nails a rough or broken appearance. herbal supplements, or vitamins. She also denied secondary to fine, superficial striations seen in a It can involve any number of nails in the upper or any or recent travel. regular, parallel pattern, giving it a sandpaper- lower limbs.1 Additionally, numerous superficial Physical exam revealed thickened dystrophic nails like appearance.1-3 The cuticle is commonly pits on nail surfaces leave the nails with a shiny with pitting, longitudinal ridging, and hyperkeratotic and ragged.3 Shiny trachyonychia, appearance. In mild cases, alternating elevations (Figure 1). The patient was diagnosed with a less common, milder variant, presents with

Figure 1

TONGDEE, SHAREEF, FAVREAU Page 43 multiple small punctate depressions that are saw-tooth acanthosis containing a band of eosinophilic onychocytes and parakeratotic cells spread in a geometric pattern within parallel lymphocytic infiltrate with vacuolar degeneration that may reside in the nail plates. The proximal lines.2 of basal keratinocytes.15 consistent nail matrix and the ventral proximal nail fold are Since the disease affects the nail matrix, a nail with psoriasis will reveal acanthosis and focal typically affected more than the distal matrix. matrix punch biopsy or longitudinal nail biopsy parakeratosis affecting the proximal nail fold This displays clinically in the dorsal nail plate. and nail matrix.15 Also, polymorphonuclear Furthermore, nail plate abnormalities are more may be performed, but this is not recommended; 18 the risk/benefit ratio is not advantageous, as the leukocytes along the whole length of the dorsal present in the dorsal layer. nail plate are commonly observed in TND procedure is invasive and the condition can be 14 Clinically, trachyonychia secondary to lichen diagnosed clinically.18 In idiopathic TND and histologically consistent with psoriasis. Focal planus or psoriasis appears identical to TND associated with alopecia areata, histology areas of nail hypergranulosis can be seen in trachyonychia due to spongiotic features.1 of the nail will most commonly demonstrate idiopathic trachyonychia, nail lichen planus However, due to the distinctive presentation spongiotic inflammation of the nail matrix and and nail psoriasis. Inflammation in the matrix of this nail plate abnormality and the risk of can affect keratinization, causing an increase exocytosis of inflammatory cells () 5 permanent nail damage from biopsy, diagnosis into the nail epithelia.1,3-5 of keratohyalin granules. Here, histology may will usually be made clinically. Trachyonychia reveal a mild to moderate lymphocytic infiltrate can often be misdiagnosed as , When the disease is caused by psoriasis or in the superficial of the proximal nail lichen planus, histology will display findings 15 so laboratory studies are recommended before fold and matrix. The ventral portion of the nail initiating antifungal therapy for the treatment of typically associated with both of these conditions. fold sometimes demonstrates 6 Histology consistent with nail lichen planus onychomycosis. Due to the many dermatological of the cuticle. Other possible findings include associations, a thorough examination looking will reveal hyperkeratosis, hypergranulosis, and longitudinal clefts containing zones of Table 1. Treatments used for TND Treatment Route Dose Time Note Steroids (general)55 Topical 1% ointment 4 months Relapse, painful, proximal nailfold, need 40 0.5 mg/kg - 1 Intralesional Bimonthly for 4 months long-term compliance (effective in 4 mg/kg 40 children) Prednisone27 PO 0.5 mg/kg Alternate days for 4 weeks No relapse

32 2 times per week for 8 Triamcinolone acetonide IM (10 mg/ml)^3 Proximal and lateral nailfolds months Mini pulse therapy (2 Shown to be effective. Usually fewer side Betamethasone58 PO 4 mg consecutive days every week effects vs. the daily dose of for 2 months) over weeks and months. Required 2 courses, with side effects of 38 peeling, on proximal nailfold Tazoretene Topical 0.10% Nightly for 3 months (showed improvement in 1 patient with alopecia areata)38 Psoriatic trachyonychia (improvement Acitretin59 PO 0.3 mg/kg Daily dose for 3 months in roughness, ridging, pitting, subungual hyperkeratosis)

42 Psoriatic trachyonychia (successful in 5 3 mg/kg/day Daily for 2.5 months patients). 42,65 Cyclosporine A PO Idiopathic trachyonychia, in case series 2 mg/kg/day -3.5 65 6 months of 15 patients 87% showed significant mg/kg/day improvement after 6 months of therapy. All treated nails showed significant 54 0.7 J/cm^2 - 1.4 J/ PUVA n/a 3 times a week for 7 months improvement, untreated remained cm^2 dystrophic.

39 Psoriatic trachyonychia; periungual 5-fluorouracil Topical 5% Every 2-4 days for 16 weeks 39,57 irritation limits the ’s use.

22-24 PO/ LP trachyonychia, general anesthesia Griseofulvin/steroid 10 mg/kg 6 months intramatrix used. Biotin43,67 PO 20 mg Daily Primary biliary cirrhosis patient.

Petrolatum63 Topical Not known Not known Partial resolution seen.

Nail plate dressings (ultra- thin adhesive layer with lactic Significant improvement at 3 months; acid, silicon dioxide, aluminum Topical Once a week 6 months near complete resolution at 6 months acetylacetonate, vinyl copolymer, azelaic acid)67

Vitamin supplement63 PO Not known Not known Partial resolution seen

Page 44 A CASE OF TWENTY NAIL DYSTROPHY AND REVIEW OF TREATMENT OPTIONS for psoriatic features, dermatosis, and/or loss was not successful and resulted in periungual should be performed. irritation that limited its use.45 Arias-Santiago Treatment of trachyonychia is aimed primarily et al. used nail plate dressings that consisted of at the underlying cause, if found.18 TND usually an ultra-thin adhesive layer containing lactic acid, remits spontaneously over several years, as it silicon dioxide, aluminium acetylacetonate, vinyl is a self-limited condition.15,18,32 Therefore, copolymer, and azelaic acid that were applied counseling and reassurance for parents and once a week for six months. This particular patients is important, as is explaining to them method showed significant improvement at three months and close to complete resolution at six that the disease is self-resolving. In about 50% 46 of patients, nail changes will reveal significant months. Mittal et al. took the unique approach improvement or resolve within five to six years.6 of administering PO 4 mg betamethasone two Waiting this long can be hard for some patients, consecutive days every week for two months. This as this condition can negatively affect quality of mini pulse therapy was shown to be effective, 7 with fewer side effects when compared to daily life. Currently, no specific treatment exists for 47 the condition, and treatment is done only for oral steroids for weeks or months. Petrolatum and vitamin supplementation showed only partial cosmetic reasons. The disease does not leave any 46,48 scars.18 resolution. Biotin was used successfully to treat TND in two patients with primary biliary The basis of treatment involves regulating the cirrhosis.46,49 differentiation of keratinocytes and/or decreasing the inflammation in the nail fold or nail matrix.33 Treating TND associated with psoriasis or lichen Conclusion TND, also known as trachyonychia, is a disease planus with systemic therapies may involve that can present independently, idiopathically, or corticosteroids, oral retinoids, or cyclosporine 3 in association with other conditions. Nail matrix (Table 1, p. 44). biopsies are usually not recommended, as they risk There have been several reports of treatment leaving the patient with permanent nail damage success. One article reported success with and the diagnosis can usually be made on a clinical griseofulvin in an intra-matrix steroid injection in basis. To date, no particular treatment has been a 6-year-old boy diagnosed with lichen planus- universally accepted. Positive results have been related TND under general anesthesia over a achieved with griseofulvin injections, PUVA, period of six months.34,35 This method has systemic steroids, oral retinoids, cyclosporine A, been quite successful for TND with associated and nail plate dressings.3 But in a large majority lichen planus.35-38 Relapse has been seen with of cases, trachyonychia is a self-limiting disease intralesional injection of , and and, as a result, treatment should only be given 39 compliance is reduced due to pain. Tosti et al. if absolutely essential, such as when the patient’s treated a lichen planus trachyonychia patient quality of life is detrimentally affected. In this with oral prednisone 0.5 mg/kg on alternate population, there are treatment options that can days for four weeks. Four months after stopping significantly shorten the duration of the disease.7,18 the treatment, the nails demonstrated only Patients should be aware of the treatment choices mild longitudinal ridging, with no relapse over and the associated risks. Patients should also be 1 three years. They also attempted intramuscular reassured that abstaining from therapy is also triamcinolone acetonide 0.5 mg/kg to 1 mg/kg an acceptable and safe option due to the benign per month in 15 children with typical nail lichen nature of the disease. planus with successful results.12 Silverman et al. treated a 9-year-old girl with lichen planus- related TND using potent steroids for six months, which did not result in any improvement.22 In psoriatic TND, recurrence of nail changes is usually seen when treatment is stopped.3 Still, a study using an oral (acitretin) 0.3 mg/ kg daily for three months showed improvement in roughness, ridging, pitting and subungual hyperkeratosis.40 Topical 0.10% retinoid (tazarotene) did not achieve the same effect, but resulted in peeling and erythema on the proximal nail folds; however, mild improvement was seen in one patient with alopecia areata.41 Two studies also showed success with oral cyclosporine A at 3 mg/kg/day: Pierard et al. administered it daily for two and half months with success in five patients with psoriatic trachyonychia,42 and a case series of 15 patients with idiopathic trachyonychia saw an 87% improvement after six months of therapy.43 When treating idiopathic trachyonychia, good results were seen with PUVA three times a week for seven months at strengths of 0.7 J/cm2 to 1.4 J/cm2.44 Topical 5-fluorouracil, on the other hand,

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