Trachyonychia Research Articles

Simultaneous occurrence of idiopathic trachyonychia in dizygotic twins.

Trachyonychia (or twenty-nail dystrophy) is an uncommon chronic disorder manifesting as thin, flattened, brittle nails with excessive longitudinal ridging and loss of luster creating a "sandpaper-like" texture that most commonly presents spontaneously in childhood as an isolated phenomenon; however, it has been historically associated with numerous dermatoses. Rarely, trachyonychia has been reported to occur in families, suggesting a potential hereditary predisposition. We report trachyonychia occurring simultaneously in dizygotic twins, further supporting a possible underlying genetic basis of this idiopathic nail disorder.

Carbamazepine-induced trachyonychia

Carbamazepine-induced trachyonychia

Twenty-Nail Dystrophy Treated with Hydroxychloroquine in a Patient with Alopecia Areata.

Twenty-Nail Dystrophy Treated with Hydroxychloroquine in a Patient with Alopecia Areata.

Rare Presentation of Alopecia Universalis Congenita and Twenty-nail Dystrophy in Siblings.

Congenital alopecia universalis is one of the rarest anomaly which involves skin and appendages. The inheritance pattern can be autosomal recessive, X-linked recessive, or autosomal dominant. However, the most common is autosomal recessive form and it is the most severe phenotype. Twenty-nail dystrophy refers to the condition in which all the twenty nails are affected in the form of excessive ridging and nail plate roughness leading to unsightly lustureless nails. We report a rare case of two siblings with alopecia universalis congenita with twenty-nail dystrophy. To the best of our knowledge, this case is the first case to be reported with such association in both siblings. This case reports highlights the fact that alopecia areata is an autoimmune disease with a genetic predisposition as in our case both siblings had alopecia universalis and nail dystrophy. There was no evidence of any other ectodermal dyplasia and had normal teeth and seat glands. The skin biopsy ruled out congenital atrichia and was suggestive of alopecia areata.

Therapeutic Efficacy of Combination Therapy Using Oral Cyclosporine with a Dietary Supplement (Pantogar®) in Twenty-Nail Dystrophy.

BackgroundTwenty-nail dystrophy (TND) is an acquired idiopathic disease characterized by dull, thin, lusterless, and fragile nails with fissuring, small regular pits, and excessive longitudinal ridging. Although various treatment modalities have been performed in order to treat TND, the effects of these treatments are controversial.ObjectiveTo evaluate the effectiveness of oral cyclosporine in TND.MethodsA total of 38 patients with TND were treated with combination therapy using oral cyclosporine with a pantothenic acid complex-based dietary supplement (Pantogar®; Merz Pharmaceuticals GmbH, Germany), whereas 44 patients were treated with the pantothenic acid complex-based dietary supplement alone. The therapeutic efficacy in each group was retrospectively evaluated using medical records and clinical photographs.ResultsThe cyclosporine therapy group had significantly more patients whose improvement was rated as almost clear, marked, or moderate compared to the control group (p<0.001). While the mean CHATS (Color, Hyperkeratosis, Area, Thickness, Separation) score of the cyclosporine therapy group was decreased by 13.45 (from 30.95 to 17.5) after treatment, the mean CHATS score of the control group was only decreased by 8 (from 29.43 to 21.43, p<0.001). Moreover, greater Dermatology Life Quality Index changes after treatment were observed in the cyclosporine therapy group (p=0.085).ConclusionOral cyclosporine can be a valuable therapeutic option in patients with TND.

Chronic Eosinophilic Leukemia Presenting Predominantly with Cutaneous Manifestations.

A 37-year-old male presented with severe oral and genital mucosal ulcers, lichenoid eruption and twenty-nail dystrophy. Systemic examination was normal, except for anemia. On investigations, he was found to have persistently elevated peripheral eosinophilia, absolute eosinophil count >5000/mm3, bone marrow showing increased eosinophilic precursors, and infiltration by atypical cells. The serum vitamin B12 levels were grossly elevated, and Philadelphia chromosome study was negative. Thus, a diagnosis of chronic eosinophilic leukemia was made. The patient showed excellent response to imatinib mesylate. We are reporting a rare type of leukemia presenting with predominantly cutaneous manifestations.

Diagnostic précoce du syndrome APECED : un défi pour le dermatologue

Polyglandular auto-immune syndrome type 1 (PAS-1) or auto-immune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a rare autosomal recessive disorder linked to auto-immune regulator (AIRE) gene mutations. Herein, we report the case of a 3-year-old boy with APECED emphasizing the wide phenotypic variability and the extent of skin lesions. A 3-year-old boy with a history of auto-immune hepatitis was referred for a generalized pruriginous urticaria-like eruption present for one month. He was born to non-consanguineous parents. Cutaneous examination revealed twenty-nail dystrophy, which had been present since the age of 2 years. Both direct microscopy and culture of nail samples were negative for Candida albicans. Esophagogastroduodenoscopy revealed esophageal candidiasis. A diagnosis of APECED was suspected and subsequently confirmed by molecular analysis of the AIRE gene, which showed two mutations. No other auto-immune endocrinopathies were found. Our case report illustrates the phenotypic variability of APECED with the absence of typical manifestations such as Addison's disease and hypoparathyroidism. APECED should thus be systematically suspected in young children presenting with cutaneous lesions associated with mucocutaneous candidiasis or auto-immune disease, even in the absence of known endocrinopathies. Dermatologists should be aware of this association since early diagnosis of APECED is critical in preventing life-threatening endocrinological crises.

Clinical study of twenty‐nail dystrophy in Korea

Twenty-nail dystrophy (TND) is an idiopathic and non-congenital nail dystrophy that is characterized by excess longitudinal ridges and loss of luster in multiple nail plates. There are a few studies on TND. In order to investigate the demographic and clinical characteristics of patients with TND in Korea, a retrospective study was performed. Eighty-eight patients were diagnosed with TND from February 1, 2003, to April 30, 2010. In this study, morphology of TND was clinically divided as atrophic, hypertrophic, pitting dominant, shallow fissure-dominant, and deep fissure-dominant types. The patients consisted of 33 males and 55 females. The proportions of subtype and gender predominance showed differences between the child-adolescent-onset TND (20 cases) and the adult-onset TND (68 cases). The clinical data of this study contribute to our understanding of TND.

Segmental vitiligo and twenty-nail dystrophy: An unusual association

Segmental vitiligo and twenty-nail dystrophy: An unusual association

Polish dermatology in the 19th and the first half of the 20th centuries

The beginnings of Polish dermatology date back to the first half of the 19th century in Kraków. The first textbook of dermatology was written by Ludwik Bierkowski. Later the progress in the development of this field of medicine was due to Franciszek Krzyształowicz, Marian Grzybowski, Franciszek Walter, and Jan Alkiewicz. Krzyształowicz's most remarkable achievements were related to his studies of the Treponema pallidum of syphilis. Grzybowski's main contribution to international dermatology was the first description in the medical literature of a specific variant of keratoacanthoma, which has since then been called Grzybowski's eruptive keratoacanthoma or generalized eruptive keratoacanthoma - Grzybowski's variant. Alkiewicz described trachyonychia, or twenty-nail dystrophy, a disease that became well established in the dermatological literature; he also described the so-called transverse net in onychomycosis. Walter identified the syphilitic skin and bone lesions in some figures carved in the Veit Stoss's altar in Kraków, thus presenting the famous thesis of the non-American origin of syphilis in Europe. Considering all these achievements, it is the goal of this paper to review Polish contributions to international dermatology.

Twenty nail dystrophy trachyonychia: An overview

Twenty nail dystrophy (TND) trachyonychia, a fascinating clinical condition, was brought to focus 25 years ago. Ever since, it has been sparingly reported. Nonetheless, the condition is well-recognized and its diagnosis is made on the basis of clinical features characterized by onset in infancy/childhood, and occasionally in adults. The lesions are fairly representative, and are characterized by the alternating elevation and depression (ridging) and/or pitting, lack of luster, roughening likened to sandpaper, splitting, and change to a muddy grayish-white color. Dystrophy is prominent. Several modes of occurrence have been described including an hereditary component. The confirmation of diagnosis is through microscopic pathology corresponding either to endogenous eczema/dermatitis, lichen-planus like or psoriasic-form. It is a self-limiting condition and may occasionally require intervention.

Twenty nail dystrophy in vitiligo

Twenty nail dystrophy (TND) is a manifestation of several dermatoses and its association with vitiligo is rare. It may be consequent upon a common autoimmune insult to the melanocytes and the nail matrix. We report a patient of acrofacial vitiligo who developed TND. Nail matrix biopsy revealed focal lichenoid reaction and chronic inflammatory infiltrate in the dermal papillae and around blood vessels. Hematological, biochemical and serological investigations showed no evidence of other autoimmune diseases.

Nail Biopsy: Assessment of Indications and Outcome

For years, nail biopsy has been shunned as a difficult and scarring procedure, which is seldom required in day-to-day practice. Only a few studies with a limited number of patients have been carried out to assess its utility in dermatology. We studied 270 patients with nail disorders (both infectious and noninfectious). In 205 cases, the clinical diagnosis could be confirmed with the help of routine diagnostic aids, in the form of potassium hydroxide preparation, fungal culture, and biopsy of associated skin lesions. In the remaining 65 cases, various types of nail biopsies were carried out after ruling out contraindications to nail surgery. Overall, the histopathologic changes were found to be diagnostic in 63% of cases. Findings were more characteristic in infectious disorders of the nail unit. The diagnostic yield varied with the type of biopsy procedure. Side effects in the form of scarring and nail dystrophy were seen in 29.2% of the patients. Nail biopsy is useful, especially in cases with isolated nail involvement, an absence of skin lesions, and disorders such as twenty-nail dystrophy. It should be advocated in cases in which the routine diagnostic procedures fail to yield results. Proper selection of cases, choice of biopsy technique, and attention to the surgical procedure help in minimizing the side effects associated with the procedure. Nail biopsy was found to be a simple, safe, and useful procedure, especially in cases in which the clinical diagnosis is otherwise obscure.

Efficacy of Triamcinolone Acetonide in Various Acquired Nail Dystrophies

The treatment of nail disorders is currently an unsatisfying exercise. Isolated nail involvement generally does not warrant any systemic therapy. At the same time, treatment is requested because of significant cosmetic and functional handicap. Intralesional triamcinolone acetonide (TA) in the proximal nail fold was evaluated as a treatment modality in 30 patients with twenty-nail dystrophy, 14 with nail lichen planus, and 6 with nail psoriasis. The number of involved nails varied from 1-20, and 1-10 nails were treated with TA. Fourteen patients discontinued treatment after 1-2 sittings. Out of the 28 patients completing the treatment protocol, 16 showed 75-100% improvement. Predominant side effects included pain, subungual hematoma formation, proximal nail fold hypopigmentation, and atrophy. TA given as a single injection in the proximal nail fold produced good improvement in a significant number of patients completing the treatment protocol. Lower concentrations of TA (5 mg/ml) were quite effective in treating various dermatoses affecting the nail unit. Our technique had fewer side effects than needle-less injection or multiple injection techniques. Careful attention to injection technique further minimized the side effects associated with the procedure. Sixteen patients completed the six-month follow-up and a relapse of nail changes was seen in 10. The relapses were equally responsive to retreatment. TA injected into the proximal nail fold area is a useful, cheap and efficacious treatment for dermatoses affecting the nail unit.

Twenty-nail dystrophy (trachyonychia) caused by lichen planus in a patient with gold allergy

Twenty-nail dystrophy (trachyonychia) caused by lichen planus in a patient with gold allergy

Ichthyosis follicularis with congenital atrichia, nail dystrophy and palmoplantar keratoderma. Variant of IFAP syndrome or a new entity?

A 23-year-old man was seen for dry, rough skin and alopecia present since birth. There was no history of impaired sweating, photophobia, or lacrimation. Examination revealed generalized cutaneous thorn-like projections with nonscarring alopecia, twenty-nail dystrophy, and palmoplantar keratoderma.

Rapp-Hodgkin syndrome

A 5-year-old boy with a history of a bifid uvula and a submucosal cleft palate presented for evaluation of brittle nails. The physical examination demonstrated cup-shaped ears, a broad nasal root, thin upper lip, mid-facial hypoplasia, coarse hair, and twenty-nail dystrophy. The clinical presentation of ectodermal dysplasia with cleft palate was consistent with Rapp-Hodgkin syndrome, which is one of several allelic diseases associated with mutations in the TP63 gene. The clinical manifestations of Rapp-Hodgkin as well as other ectodermal dysplasias with clefting are discussed.

Longitudinal nail biopsy: utility in 20-nail dystrophy.

Twenty-nail dystrophy (TND) is an inflammatory disorder of the nail unit. It is frequently encountered in childhood and is believed to be a common clinical manifestation of several nail pathologies. Varying associations and histopathologic features have been reported previously. To evaluate the clinical and histopathologic profile of TND and the utility of longitudinal nail biopsy (LNB) for the same. Thirty-two consecutive patients with TND were screened clinically. The histopathologic features of skin and longitudinal nail biopsies were also evaluated. The efficacy of nail unit biopsy was thereby assessed. Young males in the 10- to 20-year-old age group were most commonly affected (52%). Alopecia areata was the most commonly associated dermatosis (22%). LNB revealed a specific diagnosis of either psoriasis or lichen planus in 52% of the cases, whereas the rest showed nonspecific histologic changes (spongiotic trachyonychia). Scarring was a major risk associated with LNB, as evident in 28% of the patients studied. These data reveal the clinical profile and common associations of TND. They also confirm the diagnostic utility of nail biopsy in TND patients and advocate this procedure in cases in which the clinical diagnosis is otherwise obscure. However, it cannot be recommended in the routine evaluation of patients with a relatively benign disorder such as TND. Furthermore, nail biopsy is proven to be a relatively simple and useful procedure.

Funny Nails and Painful Procedures

Few pediatric patients present with nail dystrophy as a chief complaint. Primary inflammatory causes, such as psoriasis, trachyonychia (also known as

Twenty-nail dystrophy and vitiligo: A rare association

The term twenty-nail dystrophy (TND) of childhood has evolved since its initial description in 1977. TND has also been reported in adults, and many clinical as well as histopathologic associations have been described. We describe the third case of a rare association of TND with vitiligo in a 10-year-old girl. (J Am Acad Dermatol 1999;40:488-90.)