A Case of Twenty Nail 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 Medicine, 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 ****Dermatology 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 inflammation 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 retinoids, 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 nail disease in all 20 of her nails, another condition, including lichen planus (4% condition of idiopathic origin.9-12 TND has also starting with the thumb and progressing one by to 18.5% of cases), alopecia areata (45% to 83% been described in monozygotic twins. Girls and one beginning about two years earlier. She had of cases), or psoriasis (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, vitiligo, ichthyosis vulgaris, ichthyosis, presents at birth and evolves slowly, or it may with oral antifungal medications 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, alopecia universalis 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 type 1 diabetes 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 drugs 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 allergies 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 onycholysis 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 Histology 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 (lymphocytes) 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 onychomycosis, When the disease is caused by psoriasis or in the superficial dermis 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 hyperkeratosis 6 Histology consistent with nail lichen planus onychomycosis. Due to the many dermatological of the cuticle. Other possible findings include associations, a thorough skin 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 Triamcinolone 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 corticosteroids for 2 months) over weeks and months. Required 2 courses, with side effects of 38 peeling, erythema 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