Nail lichen planus in children: clinical features, response to treatment, and long-term follow-up

OBJECTIVE: To report clinical features, response to treatment, and long-term follow-up of nail lichen planus in children. DESIGN: Retrospective study involving 15 children with nail lichen planus. SETTING: Outpatient consultation for nail disorders at the Department of Dermatology of the University of Bologna, Bologna, Italy. PATIENTS OR OTHER PARTICIPANTS: We diagnosed nail lichen planus in 15 children younger than 12 years, including 10 children with typical nail matrix lesions, 2 children with 20-nail dystrophy (trachyonychia), and 3 children with idiopathic atrophy of the nails. Only 2 of the 15 children had oral lichen planus; none had cutaneous lesions. A nail biopsy confirmed the diagnosis in all cases. INTERVENTION: Intramuscular triamcinolone acetonide, 0.5 to 1 mg/kg per month, was prescribed to children with typical nail lichen planus and prolonged from 3 to 6 months until the proximal half of the nail was normal. No treatment was prescribed to patients with 20-nail dystrophy or idiopathic atrophy of the nails. RESULTS: Treatment with systemic corticosteroids was effective in curing typical nail lichen planus. Two children experienced a recurrence of the disease during the follow-up. Recurrences were always responsive to therapy. The 2 children with 20-nail dystrophy improved without any therapy. Nail lesions caused by idiopathic atrophy of the nails remained unchanged during the follow-up period. CONCLUSIONS: Nail lichen planus in children is not rare but probably underestimated. It often presents with atypical clinical features such as 20-nail dystrophy or idiopathic atrophy of the nails.     

Trachyonychia: a case report and review of manifestations,associations, and treatments

Trachyonychia ("rough nails") is best considered a reaction or morphologic pattern with a variety of clinical presentations and etiologies. It may involve only 1 or as many as 20 nails (20-nail dystrophy). It can be a manifestation of lichen planus, psoriasis, alopecia areata, immunoglobulin A deficiency, atopic dermatitis, and ichthyosis vulgaris. Nail matrix biopsy results and physical examination findings help in establishing the cause of this condition, though often trachyonychia is an isolated finding. When trachyonychia occurs in childhood as a manifestation of lichen planus, it tends to resolve with time. We review a case of trachyonychia, its association, its diagnostic evaluation, and treatment options.     

Treatment of common nail disorders

Treatment of nail diseases is not always easy; topical treatment is often ineffective because the nail plate is not penetrated by topical drugs, and the nail matrix is located under the proximal nail fold. The most frequent nail disorders are nail psoriasis and onychomycosis. In these cases, systemic treatment is often necessary to cure the nail changes. Systemic treatment with steroids is mandatory for patients with nail lichen planus because the disease may cause definitive destruction of the nail matrix. Environmental nail abnormalities are frequent and include nail brittleness, onycholysis, and chronic paronychia. Preventive measures should always be associated with treatment in these cases.     

银屑病甲的研究进展

银屑病是一种常见的易复发的慢性炎症性皮肤病,约50％的患者会出现甲改变.在关节性银屑病中,银屑病甲的发病率高达80.5％,给患者带来负担,如手指功能障碍、心理压力、社交障碍等.若银屑病患者既有甲损害又有皮肤损害,诊断较容易,但5％～ 10％的患者仅有甲改变,临床容易误诊为甲癣、甲扁平苔藓、甲营养不良等.银屑病甲的治疗较困难,需长期治疗且疗效不理想.银屑病甲的治疗可选用传统疗法或新型生物制剂疗法.银屑病甲治疗目的是减轻疼痛、减少功能障碍、缓解情绪.     

Nail Disorders in Children

Nail disorders in children can be divided into seven categories. The first is physiologic alterations, which every physician should be aware of in order to reassure parents. These usually disappear with age and do not require any treatment. Among congenital and inherited conditions, the nail-patella syndrome, with its pathognomonic triangular lunula, should not be missed as recognition of the disease allows early diagnosis of associated pathologies. The most common infection is the periungual wart, whose treatment is delicate. Herpetic whitlow should be distinguished from bacterial whitlow as their therapeutic approaches differ. Dermatologic diseases encompass eczema, psoriasis, lichen planus, lichen striatus, trachyonychia, and parakeratosis pustulosa. Lichen planus, when it presents as in adults, is important to recognize because, if not treated, it may lead to permanent nail loss. Systemic or iatrogenic nail alterations may be severe but are usually not the first clue to the diagnosis. Beau lines on several fingernails are very common in children after temperature crest. Tumors are rare in children. Radiographic examination allows confirmation of the diagnosis of subungual exostosis. Other cases should undergo biopsy. Single-digit longitudinal melanonychia in children is mostly due to nevi. Its management should be tailored on a case-by-case basis. Acute trauma should never be underestimated in children and hand surgeons should be involved if necessary. Onychophagia and onychotillomania are responsible for chronic trauma.     

Trachyonychia and related disorders: evaluation and treatment plans

Trachyonychia, or 20-nail dystrophy, is a benign nail disorder that primarily affects children. The condition may be idiopathic or associated with alopecia areata. Diagnosis of trachyonychia is based on the presence of nail roughness due to excessive longitudinal ridging or multiple small punctate depressions. One, a few, several, or all nails may be affected. The pathology shows that the disease is caused by inflammatory damage to the nail matrix due to spongiotic inflammation (72% of cases), psoriasis (10%), or lichen planus (16%). The prognosis of trachyonychia is benign, regardless of the disease which causes it. Treatment is unnecessary and often useless.     

Nail psoriasis: a review of the literature

Nails are considered epidermal appendages, and as such, are commonly affected in patients with psoriasis, 80% of whom are likely to develop nail psoriasis as a result of their condition. Two patterns of nail disorders have been shown to be caused by psoriasis. Nail matrix involvement can result in features such as leukonychia, pitting (punctures or cupuliform depressions), red spots in the lunula and crumbling. Nail bed involvement, on the other hand, can cause onycholysis, salmon or oil-drop patches, subungual hyperkeratosis and splinter hemorrhages. Nail disease causes aesthetic and functional impairment, and is indicative of more severe forms of psoriasis as well as of joint involvement. The treatment for nail psoriasis involves behavioral interventions, topical medications, or systemic therapy in case of extensive skin or joint involvement. This article presents a review of the main features of nail psoriasis, its clinical presentation, diagnostic and assessment methods, clinical repercussions, and of its available treatment options.     

Trachyonychia: a comprehensive review

Trachyonychia or rough nails, may present as an idiopathic disorder of the nails or it can be associated with other dermatological conditions. The dystrophic nail findings seen in trachyonychia are characterized by brittle, thin nails, with excessive longitudinal ridging. The most common histopathologic features associated with trachyonychia are spongiosis and exocytosis of inflammatory cells into the nail epithelia; typical features of lichen planus or psoriasis can also be detected. Determining the cause of trachyonychia is challenging. Treatment is often unsatisfactory, although in general it should be aimed at the underlying cause, if found. In most cases, the nail abnormalities improve spontaneously.     

Idiopathic trachyonychia (twenty-nail dystrophy): a pathological study of 23 patients

We report the clinical features and pathological findings in 23 patients with idiopathic trachyonychia (twenty-nail dystrophy). Clinically, the nail changes in the majority of patients consisted of the typical ‘sandapered’ appearance, with a rough, Iustreless nail plate. In some, however, the nail plate abnormality was less severe, with numerous small, superficial pits, which imparted a shiny appearance to the surface of the nail. Histology of nail biopsy specimens showed spongiotic changes in 19 patients, psoriasiform features in three, and features of lichen planus in one patient. The mean follow-up of these patients was 2 years, during which time none developed alopecia areata or mucocutaneous lesions. Idiopathic trachyonychia is therefore a consequence of several inflammatory disorders, which produce a disturbance of nail matrix kinetics. The course of the inflammation and the extent of the inflammatory process within the nail matrix produce two different patterns of nail plate surface abnormalities.     

Inflammatory nail conditions. Part 1: nail changes in psoriasis

Nail changes are visible in a variety of inflammatory dermatoses. The commonest dermatological condition with nail manifestations is chronic plaque psoriasis. This two‐part article reviews the nail signs in psoriasis in Part 1, and the nail changes in cutaneous lichen planus and alopecia areata in Part 2. It provides a brief summary of the salient points in the clinical features, management and prognosis of these entities, with practical recommendations that may be beneficial to all dermatologists.     

Nail changes in alopecia areata: an update and review

Nail changes are a common feature of alopecia areata (AA) and are a significant source of cosmetic disfigurement and functional impairment. This review provides an update of the prevalence, clinical and histopathological features, pathogenesis, differential diagnosis, clinical course, prognosis, and management of nail changes in patients with AA. Searches for peer‐reviewed journal articles were conducted using the PubMed/MEDLINE database with the search terms “nail changes alopecia areata,” “alopecia areata nails,” and specific searches on “trachyonychia alopecia areata” and “pitting alopecia areata.” Other sources of articles included the reference lists of retrieved articles. Nail changes are a common feature of AA, with an average prevalence of 30%, and can cause significant disfigurement and loss of function. Pitting and trachyonychia were by far the most common manifestations of AA, with an average prevalence of 20 and 8%, respectively. Red spotted lunulae, onycholysis, and punctate leukonychia were other reported findings. Other etiologies, such as onychomycosis or lichen planus, may coexist with or confound the diagnosis. There is limited published data on the clinical manifestations of AA‐associated nail changes and therapeutic options. Larger controlled trials are necessary to guide treatment decisions.     

Role of nail biopsy as a diagnostic tool

Nail biopsy (NB) is an investigation that is not routinely resorted to by most of the dermatologists. The commonly cited reasons are the complexity of the procedure, risk of scarring and the reluctance of the patient. However, in cases with isolated nail psoriasis, isolated nail lichen planus, onychomycosis not confirmed on direct microscopy and culture, or longitudinal melanonychia, the treating dermatologist is left with no choice but to resort to this procedure. Nail as a unit, is capable of projecting only a limited number of clinical manifestations. This is responsible for the more or less similar clinical presentation of many different nail disorders. Hence, a practical knowledge of the indications, appropriate patient selection, procedural details and histopathological interpretation of a NB is a must-have for any practicing dermatologist. The risk of scarring is none to minimal if appropriate type of biopsy is performed, not to mention the wealth of histopathological data that can be retrieved from the nail unit. This article aims to explore the various practical do's and don'ts for the NB and tells us what to expect from of the procedure.     

Nail psoriasis: a combined treatment with 8% clobetasol nail lacquer and tacalcitol ointment

Background   Nail involvement is a common and distressing feature in the course of psoriasis. Although much progress has been made in the treatment of the disease, the presence of psoriasis in the nail continues to pose a challenge. In recent years, vitamin D3 analogs and a new formulation containing 8% clobetasol-17-propionate in a colourless nail lacquer vehicle have produced good results for the control of nail psoriasis.
Objective   To determine the efficacy and safety of the combined treatment of 8% clobetasol-17-propionate in a lacquer vehicle and tacalcitol ointment in nail psoriasis.
Methods   Fifteen patients with both nail bed and nail matrix psoriasis were included in the study. They were treated with a colourless nail lacquer containing 8% clobetasol-17-propionate applied at bedtime at the weekend, and with tacalcitol ointment under occlusion on the remaining days, for 6 months.
Results   All 15 patients responded well to treatment. The therapeutic effect was very fast and directly related to the length of therapy. All nail alterations, including nail pain, were reduced, and the modified target Nail Psoriasis Severity Index fell by an average of 78% compared to baseline levels (±59.6, P  < 0.0001).
Conclusions   Combined treatment with tacalcitol ointment and 8% clobetasol-17-propionate in a nail lacquer is a safe, effective treatment for nail bed and nail matrix psoriasis.     

Non‐infectious inflammatory disorders of the nail apparatus

Inflammatory non‐infectious diseases of the nails are not uncommon. The nail changes may look different in the same diseases, but also very much alike in various different nail disorders depending on which particular structure of the nail apparatus is involved. Of all skin diseases, psoriasis is the one with the most frequent nail involvement. The presence of many pits allows the diagnosis to be made, but salmon spots and onycholysis are also characteristic features. Lichen planus may occur isolated on the nails or in association with lesions of the skin and adjacent mucosal membranes. Longitudinal striations and loss of the nail plate shine are the most frequent features of nail lichen planus. The more severe the alopecia areata is and the younger the patient is, the more often it affects the nail, usually with a rough surface and loss of shine. Eczemas and various autoimmune dermatoses may also affect the nails but the changes are not clinically specific.     

Nail plate involvement in lichen ruber planus

The authors review the literature data on the frequency of the nail plate involvement in lichen ruber planus and on the clinical features of this condition. Four patients suffering from lichen ruber planus with various manifestations of the nail plate involvement are described. The therapy efficacy depends on the severity of inflammation in the matrix unguis.     

Nail psoriasis improvement in a patient treated with fumaric acid esters

Nail psoriasis is common in adult psoriatic patients and it causes serious psychological and physical distress. Topical treatments such as corticosteroids, calcipotriol, retinoids, and 5-fluorouracil have limited efficacy and are not without side effects. Relative effective systemic treatments are ciclosporin, methotrexate and acitretin, all of which have a serious toxicity potential. Biologics in the treatment of nail psoriasis have been the subject of recent research, but their cost-effectiveness is questionable. We present a case of psoriatic nail disease which improved greatly on treatment with fumaric acid esters (FAE).     

New millennium, new nail problems

The major nail disorders in the new millennium may well be related to systemic drugs used for conditions other than nail problems. Conversely the new therapies for onychomycosis, a condition whose incidence is increasing, may result in drug interactions. We have chosen to report on the most common iatrogenic causes of nail disorders, such as toxic epidermal necrolysis, psoriasis or acral psoriasiform reaction, lichen planus or lichenoid reaction, antineoplastic therapy-induced palmar-plantar erythrodysesthesia, paronychia and pyogenic granuloma, drug-induced onycholysis and photo-onycholysis, and drug-induced scleroderma and sclerodermiform conditions. The adverse effects and drug interactions of the newer oral antifungal agents will be quoted and their management will be discussed.     

Pulsed dye laser vs. photodynamic therapy in the treatment of refractory nail psoriasis: a comparative pilot study

Background  Nail psoriasis is often refractory to traditional treatments, and patients with nail psoriasis usually demand a therapeutic option. Both photodynamic therapy (PDT) and pulse dye laser (PDL) have proved effective for plaque-type psoriasis, but they have not been evaluated in nail psoriasis. On the other hand, delta-aminolaevulinic acic has been shown to penetrate into the nail matrix and nail bed occluded with bioadhesive patches.
Objectives  To compare the efficacy of PDT and PDL in the treatment of nail psoriasis
Methods  We studied 61 nails treated with PDT and 60 nails treated with PDL in a group of 14 patients. The PDT used PDL as the light source. Sessions were applied monthly treating one hand with PDT and the other with PDL. The hand treated with PDT was occluded with methyl-aminolaevulinic acic (MAL, Metvix^®) for 3 h using a bioadhesive patch. The nails treated were evaluated at baseline, and after 3 and 6 months according to the Nail Psoriasis Severity Index (NAPSI) score.
Results  A decrease in NAPSI score was observed with both treatments and in both nail matrix and nail bed involvement. No statistical differences were found between PDT and PDL ( P =  0.632, P  = 0.084, P  = 0.535, at baseline, and 3 and 6 months, respectively), and between nail matrix and nail bed NAPSI scores ( P =  0.423 and P  = 0.853, respectively). The subjective impression of the patients was good, especially regarding the decrease in the pain.
Conclusions  PDL seems to be effective in the treatment of nail psoriasis and improves nail matrix and nail bed involvement. MAL does not seem to play role in the clinical response.

Conflicts of interest

None declared.     

甲扁平苔藓研究进展

甲扁平苔藓临床少见,可伴有皮肤扁平苔藓或独立存在.甲扁平苔藓的流行病学研究较少,其流行病学特点尚未完全阐明.甲扁平苔藓分5型:Ⅰ型为典型皮损伴有甲损害,Ⅱ型为不典型皮损伴有甲损害,Ⅲ型为头皮损害伴有甲损害,Ⅳ型为黏膜损害伴有甲损害,Ⅴ型为单纯甲损害.甲扁平苔藓有许多特征性临床表现,如翼状胬肉.临床上需与甲真菌病及其他可引起甲营养不良的疾病鉴别.甲扁平苔藓的组织病理特征与其他部位的扁平苔藓类似,皮肤镜有助于辅助诊断本病.本病治疗困难,缺少特效治疗方法.系统应用激素是唯一有循证研究支持的疗法.阿维A治疗甲扁平苔藓有效.甲氨蝶呤、环孢素等免疫抑制剂也可有效治疗甲扁平苔藓.甲扁平苔藓预后差,复发率高.