Benign or malign disturbances of the nail apparatus.

Disturbances of the nail apparatus are common and mainly benign. This review aims to investigate the aetiology of these disturbances, which range from more common benign causes to less common melanomas. Melanonychia may be the most prominent concern and is characterised by brown or black nail plate discoloration. Hence, understanding the most common nail changes, their epidemiology, pathophysiology, and clinical features are imperative to diagnosis and may prevent unnecessary surgical procedures in cases where it is not warranted.

Adult and Pediatric Nail Unit Melanoma: Epidemiology, Diagnosis, and Treatment.

Nail unit melanoma (NUM) is an uncommon form of melanoma and is often diagnosed at later stages. Approximately two-thirds of NUMs are present clinically as longitudinal melanonychia, but longitudinal melanonychia has a broad differential diagnosis. Clinical examination and dermoscopy are valuable for identifying nail findings concerning malignancy, but a biopsy with histopathology is necessary to confirm a diagnosis of NUM. Surgical treatment options for NUM include en bloc excision, digit amputation, and Mohs micrographic surgery. Newer treatments for advanced NUM include targeted and immune systemic therapies. NUM in pediatric patients is extremely rare and diagnosis is challenging since both qualitative and quantitative parameters have only been studied in adults. There is currently no consensus on management in children; for less concerning melanonychia, some physicians recommend close follow-up. However, some dermatologists argue that the "wait and see" approach can cause delayed diagnosis. This article serves to enhance the familiarity of NUM by highlighting its etiology, clinical presentations, diagnosis, and treatment options in both adults and children.

Risk of recurrence of nail unit melanoma after functional surgery versus amputation.

BACKGROUND: Minimally invasive nail unit melanoma (NUM) can be treated with functional surgery (FS) instead of amputation. OBJECTIVE: To determine risk factors associated with recurrence in NUM. METHODS: We retrospectively reviewed patients with NUM between 2008 and 2022 at a tertiary referral center. Multivariable Cox regression models adjusted for male sex and Breslow thickness (BT) were generated. Receiver operating characteristic analysis was performed to determine optimal cut-off points of the BT for stratifying recurrence risk. RESULTS: We evaluated 140 NUM cases (33 amputation and 107 FS). The mean BT values were 3.14 ± 2.62 mm (amputation) and 0.70 ± 1.36 mm (FS). Recurrence occurred in 10 (30.30%) patients with amputation and 23 (21.5%) with FS. Distant disease occurred in 10 (30.30%) patients with amputation and 8 (7.48%) with FS. Male sex, greater BT, amelanotic color, ulcers, and nodules were associated with greater risk for recurrence or distant disease. A BT of 0.8 mm was deemed the optimal cut-off for stratifying recurrence risk after surgery (odds ratio, 5.32; 95% CI, 2.04-13.85). LIMITATIONS: Small sample. CONCLUSION: FS can be considered for NUM with a BT < 0.8 mm, providing an amputation-sparing benefit. However, NUM with risk factors for recurrence requires patient counselling and close follow-ups.

The impact of nail psoriasis on disease activity, quality of life, and clinical variables in patients with psoriatic arthritis: A cross-sectional multicenter study.

AIM: Nail involvement is common in psoriatic arthritis. This study assesses clinical characteristics, nail psoriasis prevalence, and impact of nail psoriasis on disease activity in patients with psoriatic arthritis (PsA). METHOD: This cross-sectional multicenter study was conducted by the Turkish League Against Rheumatism using PsA patients recruited from 25 centers. Demographic and clinical characteristics of PsA patients, such as disease activity measures, quality of life, and nail involvement findings were assessed during routine follow-up examinations. Patients were divided into two groups according to the presence or absence of nail psoriasis and compared using the χ2 test or Fisher exact test for categorical variables and the t-test or Mann-Whitney U test for continuous variables. RESULTS: In 1122 individuals with PsA, 645 (57.5%) displayed nail psoriasis. The most frequent features of fingernails were ridges (38%), followed by pitting (21%) and onycholysis (19%). More females were present in both groups (with and without nail psoriasis; 64% vs 67%, P < 0.282). Patients with nail psoriasis were older, indicated more pain and fatigue, experienced greater swelling, tender joint counts, and skin disease severity, and had a higher disease activity score compared with those without nail psoriasis (all P < 0.05). CONCLUSION: We demonstrate an increased prevalence of nail psoriasis observed in patients with psoriatic arthritis. Patients with nail involvement experience increased disease activity, lower quality of life, and diminished mental and physical status compared with those without nail involvement.

Clinico-epidemiological profile and management outcome of subungual digital glomus tumor-Indian experience.

BACKGROUND: Glomus tumors are rare tumors arising from the mesenchymal smooth muscle cells of the glomus body. They are extremely painful tumors but because of their subungual location, remain mostly underdiagnosed. AIM: To characterize the demographic, clinical, onychoscopic, radiological features and management outcome of subungual glomus tumor. Material & methods- 15 patients with a total of 16 subungual glomus tumors were evaluated and their demographic data, history, clinical features, investigations, treatment, and follow-up were analyzed. RESULTS: Glomus tumors had a female preponderance (11/15) with thumb being the commonest site. All patients presented with intractable pain. Nail discoloration was observed in 11/16 (68.8%) lesions and nail plate deformity in 6/16 (37.5%) lesions. Common features on onychosocpy were pink glow and linear vascular structures. Doppler sonography and/or magnetic resonance imaging confirmed the diagnosis of glomus tumor in all the lesions. Surgical excision was done under local anesthesia using a trans-ungual approach and no recurrence was seen. CONCLUSIONS: High index of suspicion, meticulous clinical assessment along with radiological investigations can help in the early diagnosis. Complete surgical excision is the treatment of choice to prevent recurrence.

Subungual Melanocytic Lesions in Pediatric Patients. / Lesiones melanocíticas subungueales en la edad pediátrica.

The study of subungual melanocytic lesions can present challenges because of the clinical and histologic characteristics of the nail unit and the difficulty of performing nail biopsies and processing specimens. These lesions can be even more challenging in children due to differences in clinical and epidemiological profiles between the adult and pediatric populations. Many of the clinical features of subungual melanocytic lesions that would raise alarm in an adult do not have the same implications in children. Consensus is also lacking on when a nail biopsy is needed to rule out malignancy in the pediatric setting. In view of these considerations and the rarity of subungual melanoma in childhood, the recommended approach in most cases is a watch-and-wait strategy. Subungual melanocytic lesions in children may also show atypical histopathologic features that are not necessarily associated with aggressive behavior. Subungual melanoma is very rare in childhood, with just 21 cases described to date. None of the patients developed visceral metastasis or died as a result and the diagnosis was controversial in many of the cases. Considering the above and the significantly higher frequency and particular characteristics of longitudinal melanonychia with a benign etiology in children, subungual melanocytic lesions should be managed differently in this setting than in adults. In most cases, a watch-and-wait approach is the most appropriate strategy.

Clinical features and natural course of pediatric longitudinal melanonychia: A retrospective cohort study in Korea.

BACKGROUND: Large studies on the clinical features and natural course of pediatric longitudinal melanonychia (LM) are lacking. OBJECTIVE: To investigate the clinical features and natural course of pediatric LM. METHODS: Retrospective cohort analysis of pediatric patients (age ≤ 18 years) with LM. RESULTS: We examined 703 LM lesions in 381 children. Single, narrow, and homogeneously pigmented fingernail lesions were most frequently observed. Our results suggested that within 3, 4.5, and 9.5 years after onset, approximately 3%, 5%, and 10% of LM lesions, respectively, will completely regress and that single, left-sided, and homogeneously pigmented lesions are more likely to disappear completely. The age of onset, sex, finger/toe position, Hutchinson's sign, and nail dystrophy were not associated with complete regression. During follow-up, most cases demonstrated no change in color or width between the first and last visit, and early darkening/widening before stabilization or lightening/narrowing was common. The lightning of pigmentation was associated with complete regression, whereas change in width was not. LIMITATIONS: Retrospective study at a tertiary center. CONCLUSION: Our results suggest that clinicians ought to follow pediatric patients with LM without intervention for several years even if lesions grow darker or wider. Single, left-sided, and homogeneously colored lesions are more likely to regress.

Hand-foot syndrome and nail disorders secondary to treatment with paclitaxel: Is there a relationship with the presence of fungi?

INTRODUCTION: This study aimed to evaluate the frequency of nail disorders and the presence of fungi on the nails of the hands and feet of patients with hand-foot syndrome secondary to treatment with paclitaxel. METHODS: Prospective study, carried out from October 2018 to December 2019, which included 81 patients undergoing treatment for breast cancer using paclitaxel and had signs and or symptoms of hand-foot syndrome with or without nail disorders. The data were collected through interviews guided by a structured questionnaire, information from medical records and reports of mycological exams. RESULTS: The average age of women was 54.7 ± 7.4 years. Nail disorders occurred in 69 patients (85.2%), and of these, 43 (62.3%) were positive for fungi. The fungi were yeasts (n = 38; 69%), dermatophytes (n = 15; 27.2%) and non-dermatophyte filamentous fungi (n = 8; 14.5%). CONCLUSIONS: Nail disorders were the most frequent manifestations in patients with hand-foot syndrome treated with paclitaxel and occurred in 85.2% of them. It was evidenced that fungi are present on the nails of these patients and can occur in up to 65.28%. The most prevalent fungi were Candida and Trichophyton. The nail lesion was associated with the type of treatment protocol used by the patient. The results of the study point to the need to select safe management alternatives for patients, so they can prevent nail lesions and prevent the proliferation of fungi, consequently reducing negative life impact during treatment.

Epidemiological analysis of nail aspergillosis and non-dermatophyte moulds. Criteria for the involvement of Aspergillus in 102 cases of onychomycosis at Montpellier University Hospital, France (1991-2019).

BACKGROUND: Moulds are often wrongly considered contaminants, not very sensitive to conventional antifungal treatments, but they may cause ungual hyphomycosis, particularly Aspergillus. Due to the lack of precise diagnostic criteria, their real impact is underestimated. OBJECTIVES: Retrospective descriptive analysis of all ungual hyphomycosis cases diagnosed at Montpellier Hospital from 1991 to 2019 to: (i) determine the incidence of onychomycosis by pseudo-dermatophytes and moulds; (ii) perform an epidemiological analysis of nail aspergillosis; and (iii) provide simple criteria for mould involvement in onychopathy. PATIENTS/METHODS: Data concerning 4053 patients were collected: age, sex, onychomycosis location, direct examination results, species(s) identified and fungal co-infections. Moreover, clinical data of patients with nail aspergillosis were analysed to identify potential contributing factors, and the classical criteria for mould involvement in onychopathy were critically reviewed. RESULTS: A pseudo-dermatophyte or a mould was involved in nail colonisation in 17.25% of patients (men/women ratio: 0.70; mean age: 53.1 years). The identified hyphomycetes belonged mainly to the genera Fusarium (45.68%), Scopulariopsis (30.23%) and Aspergillus (16.94%). Analysis of the clinical reports of 102 patients with ungual aspergillosis (men/women ratio: 0.67; mean age: 56.3 years) identified cardiovascular (43.9%), endocrine (25.8%), cancer (19.7%) and skin (18.2%) diseases as contributing factors. CONCLUSIONS: The adoption of simple and reliable criteria (ie, characteristic filaments on direct microscopic examination after periodic acid-Schiff staining, growth at seeding points in culture) allows determining the formal involvement of a mould in chronic onychopathies and avoiding possible side effects and costs of empirical or inappropriate and repetitive treatments.

Challenge of Nail Psoriasis: An Update Review.

Nail psoriasis is a refractory disease that affects 50-79% skin psoriasis patients and up to 80% of patients with psoriatic arthritis (PsA). The pathogenesis of nail psoriasis is still not fully illuminated, although some peculiar inflammatory cytokines and chemokines seems to be the same as described in psoriatic skin lesions. Psoriatic nail involving matrix can cause pitting, leukonychia, red spots in lunula, and nail plate crumbling, while nail bed involvement can result in onycholysis, oil-drop discoloration, nail bed hyperkeratosis, and splinter hemorrhages. The common assessment methods of evaluating nail psoriasis includes Nail Psoriasis Severity Index (NAPSI), Nail Assessment in Psoriasis and Psoriatic Arthritis (NAPPA), Nail Psoriasis Quality of life 10 (NPQ10), and so on. Treatment of nail psoriasis should be individualized according to the number of involving nail, the affected site of nail and presence of skin and/or joint involvement. Generally, topical therapies are used for mild nail psoriasis, while biologic agents such as etanercept are considered for severe nail disease and refractory nail psoriasis. Even though the current literature has shown some support for the pathogenesis, clinical presentation, or therapies of nail psoriasis, systemic review of this multifaceted disease is still rare to date. We elaborate recent developments in nail psoriasis epidemiology, pathogenesis, anatomy, clinical manifestation, diagnosis, differential diagnosis, and therapies to raise better awareness of the complexity of nail psoriasis and the need for early diagnosis or intervention.

Association of Terry Nails With Liver Cirrhosis.

INTRODUCTION: We looked at the association between Terry nails and liver cirrhosis in an ambulatory population from hepatology and gastroenterology clinics. METHODS: We prospectively investigated the prevalence and determinants of Terry nails in 1,000 consecutive patients from hepatology and gastroenterology clinics at 2 institutions between May 2016 and February 2020. RESULTS: A total of 117 subjects manifested Terry nails, with a 25.6% prevalence in patients with cirrhosis. When adjusted for age, heart failure, diabetes mellitus type 2, and chronic liver disease, cirrhosis was the only significant correlate (odds ratio 5.7 [95% confidence interval 3.3-9.8]), irrespective of liver disease etiology, with a strong association with hepatic fibrosis stage (P < 0.0001). DISCUSSION: Sensitivity and specificity of Terry nails for cirrhosis (25.8%, 92.7%) was similar to palmar erythema but less than spider angioma.

Identification of clinically useful predictive genetic variants in pachyonychia congenita.

BACKGROUND: Pachyonychia congenita (PC) refers to a group of autosomal dominant disorders caused by mutations in five keratin genes (KRT16,KRT6A,KRT17,KRT6B or KRT6C). Current disease classification is based on the gene harbouring disease-causing variants. AIMS: We harnessed the International Pachyonychia Congenita Research Registry (IPCRR) containing both clinical and molecular data on patients with PC worldwide, to identify genetic variants predicting disease severity. METHODS: We ascertained 815 individuals harbouring keratin mutations registered in the IPCRR. We looked for statistically significant associations between genetic variants and clinical manifestations in a subgroup of patients carrying mutations found in at least 10% of the cohort. Data were analysed using χ2 and Kruskal-Wallis tests. RESULTS: We identified five mutations occurring in at least 10% of the patients registered in the IPCRR. The KRT16 p.L132P mutation was significantly associated with younger age of onset, presence of palmar keratoderma oral leucokeratosis and a higher number of involved nails. By contrast, the KRT16 p.N125S and p.R127C mutations resulted in a milder phenotype featuring a decreased number of involved nails and older age of onset. Patients carrying the p.N125S mutation were less likely to develop palmar keratoderma while p.R127C was associated with an older age of palmoplantar keratoderma onset. Moreover, the KRT17 p.L99P mutation resulted in an increased number of involved fingernails and patients demonstrating 20-nail dystrophy, while the opposite findings were observed with KRT17 p.N92S mutation. CONCLUSIONS: We have identified novel and clinically useful genetic predictive variants in the largest cohort of patients with PC described to date.

Toxicity profile of taxanes in Tunisian cancer patients: A retrospective study of 90 cases.

INTRODUCTION: Taxanes are widely used in medical oncology. The aim of our study was to report and analyze the toxicity features of these drugs in Tunisian patients and to determine their impact on treatment response. METHODS: Our retrospective study concerned 90 patients treated by taxanes in a medical oncology unit, from January 2014 to January 2017. We collected their epidemiologic and anatomo-clinical data and we detailed toxicity features including types grades and impact on tumor response. RESULTS: Median age was 46 years. 80% of patients had breast cancer. Tumors were metastatic in 23.3% of cases. Nail toxicity was observed in 100% of patients. Grade I-II digestive toxicity was observed in 54.4% of cases. Hematological toxicity was noted in 42.2% of patients and it reached grade III-IV in five patients. Neurological toxicity occurred in 31% of patients and was grade III-IV in 6 cases. Alopecia was observed in 60% of patients. Fatigue was noted in 57.8% of patients. Myalgia was observed in 42.2% of patients. Toxicity did not affect the response to treatment. CONCLUSION: The taxanes' toxicity profile in Tunisian patients is characterized by more frequent digestive and nail toxicities and less frequent hematological toxicities, dose reduction and treatment delays than other populations.

Prevalence of extra-articular manifestations in psoriatic arthritis: a systematic review and meta-analysis.

OBJECTIVE: To describe the prevalence of extra-articular manifestations-enthesitis, dactylitis, nail disease, uveitis and IBD-in PsA, and their impact on longitudinal disease outcomes. METHODS: We searched Medline, PubMed, Scopus and Web of Science using a predefined protocol in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Studies using imaging to define extra-articular manifestations (EAMs) were excluded. Where possible, we performed meta-analyses of prevalence estimates, reported as percentages (95% CI). Heterogeneity (I2 statistic) was examined according to study characteristics. RESULTS: We identified 65 studies amounting to a total of 163 299 PsA patients. Enthesitis was assessed in 29 studies with an average prevalence of 30% (95% CI: 24%, 38%). Dactylitis was reported in 35 studies with an average prevalence of 25% (95% CI: 20%, 31%). Nail disease was present in 60% (95% CI: 52%, 68%) across 26 studies, but definitions were often unclear. Uveitis (3.2%; 95% CI: 1.9%, 5.3%) and IBD (3.3%; 95% CI: 1.5%, 7.1%) were less common. Heterogeneity was high (>95%) in all meta-analyses, but could not be explained by study characteristics. No studies examined the impact of EAMs on longitudinal disease outcomes, except that dactylitis increases radiographic progression. CONCLUSION: Enthesitis, dactylitis and nail disease are highly prevalent in PsA, but not uveitis and IBD. EAM patterns differ from axial SpA despite their shared disease mechanisms, which may help further understand differences between spondyloarthritides. More studies are needed on the impact of EAMs on disease outcomes such as response to treatment.