Herramientas de medición de la patología ungueal: revisión literaria / Measuring tools for nail pathology: literature review

El sistema ungueal conforma una compleja unidad anatómica fundamental en el ser humano, susceptible de sufrir numerosas alteraciones debido a procesos patológicos, tanto sistémicos como locales. El estudio de la patología ungueal es un tema de especial interés, sin embargo, en el ámbito clínico todavía resulta complicado analizar parámetros como área concreta de extensión, anatomía comprometida o porcentaje de afectación, dificultando poder realizar una valoración objetiva y establecer pautas efectivas de tratamiento, debiendo recurrir, en numerosas ocasiones, al examen histopatológico. En este sentido, los índices de medición se posicionan como una herramienta que permitiría valorar estos factores de forma numérica, reproducible y simple. El objetivo de este estudio fue identificar las herramientas de medición de las diferentes patologías ungueales descritas en la literatura. Se realizó una revisión de la literatura por pares en cuatro bases de datos atendiendo a criterios del Critical Appraisal Skills Programme España (CASPe). Los hallazgos describieron la existencia de 12 herramientas de medición de patología ungueal. Las conclusiones señalaron, finalmente, el Nail Psoriasis Severity Index o índice NAPSI como método más utilizado para valorar la severidad de la psoriasis ungueal, pese a los reportes obtenidos en sus modificaciones posteriores. En cuanto a la onicomicosis, el Scoring Clinical Index for Onychomycosis o índice SCIO resultó ser el método objetivo más útil en la práctica clínica. Para la paroniquia crónica, el Índice de paroniquia crónica se distinguió como una buena alternativa a las opciones de valoración previas (AU)

The nail system forms a complex anatomical unit fundamental in humans, susceptible to numerous alterations due to both systemic and local pathological processes. The study of nail pathology is a subject of special interest, however, in the clinical setting, it is still difficult to analyse parameters such as specific area of extension, anatomy involved or percentage of involvement, making it difficult to carry out an objective assessment and establish effective treatment guidelines, having to resort, on numerous occasions, to histopathological examination. In this sense, measurement indices are positioned as a measurement tool that would allow these factors to be assessed in a numerical, reproducible and simple way. The aim of this study was to identify measurement tools for the different nail pathologies described in the literature. A peer-review of the literature was conducted in four databases according to Critical Appraisal Skills Programme España (CASPe) criteria. The findings described the existence of 12 nail pathology measurement tools. Conclusions finally pointed out the Nail Psoriasis Severity Index or NAPSI index as the most widely used method to asses the severity of nail psoriasis, despite reports of subsequent modifications. For onychomycosis, the Scoring Clinical Index for Onychomycosis or SCIO index appeared to be the most useful objective method in clinical practice. For chronic paronychia, the Chronic Paronychia Index standed out as a good alternative to previous assessment options (AU)

Squamous Cell Carcinoma of the Nail Unit: A Clinical Histopathologic Study and a Proposal for Classification.

BACKGROUND: There is no consensus on the classification, grading, and the treatment of nail squamous cell carcinoma (NSCC). OBJECTIVE: The aim of the study was to propose a possible classification of NSCC. MATERIALS AND METHODS: Nail squamous cell carcinoma referred from January 2006 till December 2014 was included. On the basis of the clinical presentation, patients with NSCC were divided in 2 groups. Group A tumors presented as nodular or ulcerated masses of the nail bed, whereas Group B tumors presented as hyperkeratotic bands. In these 2 groups, differences in proportions (sex, histopathologic grading, and treatment performed) were evaluated with the chi-square test. RESULTS: Forty-one NSCCs were included. The groups of NSCC differed regarding: (1) the age of the patients, (2) histopathology, and (3) surgical approach. CONCLUSION: Nail squamous cell carcinomas may originate from 2 different epithelia, presenting a diverse clinical behavior. The correct identification and diagnosis of each subgroup of NSCC could be helpful in standardizing management of this tumor. Future studies considering human papillomavirus subtyping and including a major number of tumors should be performed to confirm or reject the authors' hypothesis. LIMITATIONS: This is a retrospective study, presenting the data and the experience of a single institute.

Patología de las uñas en la infancia. Cómo interpretar los cambios en la superficie ungueal / Nail alterations in children. How to interpret the changes in the ungueal surface

La patología ungueal en la infancia es muy amplia y su conocimiento es imprescindible para el diagnóstico de variados procesos que muestran en las uñas su seña de identidad propia. En muchos casos la afectación ungueal es la pionera de la enfermedad, permitiendo un diagnóstico precoz. A nivel de la atención pediátrica extrahospitalaria, el conocimiento de la semiología ungueal permite orientaciones diagnósticas en las que no es preciso el uso de complejas y costosas técnicas complementarias. Revisaremos los cambios en la superficie de la lámina ungueal (cambios en la lisura, curvatura, grosor...) y su implicación clínica, mostrando especial interés en recalcar los procesos dermatológicos o sistémicos que acompañan a cada síntoma ungueal

Ungueal pathology in children is very extensive and its knowledge is essential to diagnose varied processes that leave an identifying mark on the nails. In many cases, the affected nail is the pioneer of the disease, allowing for an early diagnosis. In regards to outpatient pediatric care, knowledge of nail semiology allows for diagnostic orientations in which the use of complex and costly complementary techniques is not necessary. We review the changes on the nail plate surface (changes in smoothness, curvature, thickness, etc.) and its clinical implication, showing special interest in emphasizing the dermatological or systemic processes that accompany each ungueal symptom

Psoriasis and associated variables in classification and outcome of juvenile idiopathic arthritis - an eight-year follow-up study.

BACKGROUND: To study the impact of psoriasis and features associated with psoriasis on classification and outcome in a population-based follow-up cohort of children with juvenile idiopathic arthritis (JIA). METHODS: In all, 440 children with JIA were followed for a median of 8 years in a prospective Nordic population-based cohort study. Data for remission was available for 427 of these children. The presence of psoriasis, psoriasis-like rash, dactylitis, nail pitting, enthesitis, tenosynovitis and heredity was assessed in relation to ILAR classification and remission. RESULTS: Clinical findings associated with psoriasis developed consecutively during the 8-year period. Six of 14 children with psoriasis were not classified as juvenile psoriatic arthritis according to the ILAR criteria at 8 year follow-up. Dactylitis was more common in children with early onset of JIA. After 8 years we found a cumulative median number of eleven arthritic joints in children with psoriasis or psoriasis-like rash compared with six in the rest of the cohort (p = 0.02). Also, the chance for not being in remission after 8 years increased significantly in patients with psoriasis, psoriasis-like rash or at least two of: 1) first-degree heredity for psoriasis or psoriatic arthritis, 2) dactylitis or 3) nail pitting, compared with the rest of the group (OR 3.32, p = 0.010). CONCLUSIONS: Our results indicate a more severe disease over time in psoriasis-associated JIA, as features of psoriasis develop during the disease course. This group is a major challenge to encompass in a future JIA classification in order to facilitate early tailored treatment.

The relationship between quality of life and the severity of psoriasis in Turkey.

BACKGROUND: Patients with psoriasis experience a low quality of life and high treatment burden: OBJECTIVES: To assess psoriatic patient quality of life using the Dermatology Life Quality Index (DLQI) in the Northeastern Anatolia region of Turkey. Additionally, we evaluated the correlation between the DLQI and the clinical severity of psoriasis and examined demographic data and their relationship with the DLQI and psoriasis severity: MATERIALS AND METHODS: This study was a single-center, prospective, cross-sectional study at the University of Kafkas, Kars, Turkey. 127 adult patients were included in the study. The Turkish version of the DLQI was used. To measure psoriasis severity, the Psoriasis Area Severity Index (PASI) and Body Surface Area (BSA) were simultaneously evaluated. The patient demographics were compared with quality of life and the severity of psoriasis: RESULTS: DLQI scores ranged from "very large" to "extremely large" in 61% of the patients. The psoriasis severity (BSA and PASI) was "mild" in 63% of patients. The quality of life was significantly affected in cigarette smokers and in patients whose disease included nail involvement. The PASI and BSA scores of patients with scalp and nail involvement were significantly higher. A significant, positive correlation was found between disease duration and the severity of psoriasis. BSA correlated with PASI: CONCLUSION: The quality of life of psoriasis patients is strongly reduced. A significant relationship was found for DLQI with nail psoriasis and smoking. A linear, positive correlation was detected between the DLQI and BSA but not between the DLQI and PASI.

Proposed classification of longitudinal melanonychia based on clinical and dermoscopic criteria.

BACKGROUND: For longitudinal melanonychia, clinical and dermoscopic criteria for differentiating malignant melanoma in situ from benign nevus/lentigo/functional melanonychia have not been fully established. OBJECTIVE: To propose a clinical classification of longitudinal melanonychia that is useful in judging the need for follow-up. METHODS: A total of 137 patients with longitudinal melanonychia referred to our outpatient clinic in the most recent eight years were included. The mean and median lengths of follow-up for patients were 5.0 and 5.5 years, respectively. We classified the 137 lesions into three types by clinical and dermoscopic features of the nail and periungual skin, including Hutchinson sign, variation of color, and borders in the pigmentation band. We observed type I and II lesions with dermoscopy every six months and three months, respectively. RESULTS: After follow-up, all 72 lesions classified as type I were thought to be benign nevus/lentigo/functional melanonychia. Five of the 52 lesions classified as type II showed enlargement during follow-up, and biopsy was performed. Of these five lesions, three were diagnosed as nevus/lentigo, and the other two were diagnosed as malignant melanoma in situ. All 13 lesions classified as type III were diagnosed as malignant melanoma in situ. CONCLUSION: We can expect a type I lesion to be a benign nevus/lentigo/functional melanonychia and a type III lesion to be a malignant melanoma in situ; however, type II lesions fall in a gray zone. We believe this classification is useful in deciding treatment and follow-up.

[Diseases of the nails]. / Krankheiten der Nägel.

Nail alterations are frequently seen in daily practice, but they are often difficult to interpret. Basic knowledge of the anatomy and biology of the nail facilitates their diagnosis as this frequently allows their development and morphology to be explained. The following short review gives hints at the most important infections, inflammatory nail diseases and tumors.

Onychocytic carcinoma: a new entity.

We have recently described a new nail tumor known as onychocytic matricoma. Herein, we describe its malignant counterpart. Clinically, the tumor simulates onychomatricoma (OM). Histologically, this in situ malignant epithelial tumor exhibits a distinct picture of onychocytic differentiation with signs of both nail matrical differentiation and nail plate differentiation. We have proposed the name onychocytic carcinoma for this singular adnexal neoplasm. Given the peculiar thickening of the nail plate observed in OM, onychocytic matricoma, and onychocytic carcinoma, the clinical individualization of a new type of nail band pattern could be proposed. It presents as an acquired localized (monodactylous) longitudinal pachyonychia. Such longitudinal pachyonychia allow the recognition of the matrical nail tumor, which has a limited etiological spectrum. Xantholeucopachyonychia suggests mainly OM and rarely onychocytic carcinoma. Pachymelanonychia suggests onychocytic matricoma and rarely pigmented OM or onychocytic carcinoma.

Hypopigmented onychocytic matricoma as a clinical mimic of onychomatricoma: clinical, intraoperative and histopathologic correlations.

Onychocytic matricoma is a newly described matrical tumor of the nail unit that clinically presents with localized thickening of the nail plate and melanonychia and represents a benign acanthoma of onychocytes. Onychocytic matricoma can be classified according to its histopathologic type (acanthotic, papillomatous or keratogenous with retarded maturation) and pigmentation (pigmented, melanocytic or non-pigmented). However, there are no published reports of non-pigmented onychocytic matricoma. We report a case of hypopigmented onychocytic matricoma that presented with a thickened nail plate, xanthonychia and histopathologic features of acanthosis, prekeratogenous zone and keratogenous zone cells forming pseudosquamous eddies, and minimal pigmentation with Fontana staining. We also provide detailed clinical, intraoperative and histopathologic correlations of this rare tumor. Both clinicians and dermatopathologists should be aware that onychocytic matricoma can present with xanthonychia, thickening of the nail plate and mimic an onychomatricoma.

[Diseases seen during a hospital consultation of nail diseases: prospective study]. / Motifs de consultation thématisée de maladie unguéale : étude prospective.

OBJECTIVE: Purpose of our study was to specify type of diseases seen during a hospital consultation of nails diseases. In our knowledge, no study on the subject was until published. METHODS: Study was realized in a forward-looking way during a period of 1 year between 13/09/2007 and 18/09/2008. It was realized in dermatology department at Orleans, during the consultation specialized in nail pathology (weekly vacation). RESULTS: One hundred and thirty-six patients were included in the study: 60.3% (82) was women. Average age was 47.9 years (extremes: 8-92 years). In all, 24.3% (33) of patients were sent by dermatologist, 8.1% (11) by hospital doctor, 2.1% (3) by pedicurist. Nail diseases observed were: ingrowing toenails: 36.8% (50 patients), onychomycoses: 31.6% (43 patients), traumatic or frictional nail lesions: 11.7% (16 patients), longitudinal mélanonychies: 8.1% (11), benign tumors: 5.1% (7 patients), nail psoriasis: 5.1% (7 patients), lichen: 1.5% (2 patients). DISCUSSION: Patients seen during this consultation were of all age, with a clear feminine ascendancy (probably connected to aesthetic embarrassment). A significant number of patients were sent by dermatologist (24.3%) or hospital doctor (8.2%): it is a consultation of expertise. Ingrowing toenails and onychomycoses were the most frequent motives for consultation, these correspond to nail pathologies the most frequent in general population.

Management of simple brittle nails.

Brittle nails are characterized by roughness of the surface of the nail plate, fragility, and peeling. This is a condition that is seen commonly in elderly individuals, and it is associated mostly with an abnormality of keratin, keratin associated proteins, water, and/or lipid content. It is idiopathic in the majority of the cases, but there are some dermatological and systemic diseases that can be associated with brittle nails. Treatment is a challenge for the clinician, in some case, can be difficult, and certainly needs strong cooperation of the patient.

Las uñas en la lepra / Nail Involvement in Leprosy

La lepra es una enfermedad causada por Mycobacterium leprae. Afecta principalmente a los nervios y a la piel, y hasta en tres de cada cuatro pacientes también a las uñas. Las causas desencadenantes de las lesiones ungueales en la lepra son múltiples, y de ellas destacan los traumatismos repetidos, la neuropatía, la insuficiencia vascular, las infecciones, las leprorreacciones o los fármacos utilizados en el tratamiento. Entre los cambios más destacados se encuentran los hematomas subungueales, la onicolisis, la onicauxis, la onicogrifosis, el pterigium unguis dorsal o la onicoheterotopia, y en su mayoría pueden atribuirse al daño nervioso y a los traumatismos. Por otro lado, la acrosteolisis que se produce en estadios avanzados puede cursar con braquioniquia, uñas en raqueta o incluso llegar a la anoniquia. Las infecciones de las uñas, con la aparición de paroniquia y onicomicosis, constituyen otro de los capítulos a tener en cuenta en la lepra. Además hay otras alteraciones caracterísiticas como las estrías longitudinales, los pits, la macrolúnula, las uñas de Terry, la leuconiquia, la hapaloniquia o las líneas de Beau. A lo largo de esta revisión se describen los principales cambios que se producen en las uñas por esta enfermedad, que son muy variados y de origen muy diverso, y de hecho son el reflejo de la amplia morbilidad que causa la infección por M. leprae (AU)

Leprosy, a disease caused by Mycobacterium leprae, primarily affects the skin and nerves, but the nails are also involved in as many as 3 out of 4 patients .The factors that trigger nail changes in leprosy are numerous and include repeated trauma, neuropathy, vascular impairment, infections, lepra reactions, and the drugs used to manage the disease. The changes most often reported include subungual hematomas, onycholysis, onychauxis, onychogryphosis, pterygium unguis, and onychoheterotopia, most of which can be attributed to nerve damage and trauma. Furthermore, the acro-osteolysis that occurs in the advanced stages of the disease may present with brachyonychia, racquet nails, or even anonychia. Infections of the nail bed leading to paronychia and onychomycosis should also be taken into account in leprosy. Other typical changes include longitudinal striae, pitting, macrolunula, Terry nails, leukonychia, hapalonychia, and Beau lines. In this review, we describe the principal nail changes associated with leprosy. These changes, which are highly varied and diverse in origin, are in fact a reflection of the significant morbidity caused by M leprae infection (AU)

"Onycholemmal carcinoma." An unusual case with apocrine and sebaceous differentiation. Are these tumors a microcystic nail bed carcinoma?

Subungual malignant epithelial tumors with tricholemmal keratinization have rarely been described as "malignant proliferating onycholemmal cyst" and "onycholemmal carcinoma (OC)." We report an additional case of a slow growing OC occurring on the middle finger of a 58-year-old man, which was unusual as it showed sebaceous-apocrine differentiation, in addition to a nail bed carcinoma with tricholemmal microcysts. We therefore consider the descriptive term of microcystic nail bed carcinoma more appropriate than OC. It is recognized that none of the rare cases of OC meet the classical additional criteria proposed by Headington for tricholemmal carcinoma, that is, lobular arrangement, peripheral palisading, thickened basement membrane, and glycogen-positive tumors cells. On the other hand, we suggest that the term follicular microcysts of the nail bed should be retained to describe the true nature of subungual epidermoid inclusions, which show usually a limited differentiation toward the follicular isthmus. Therefore, the previous cases of OC without sebaceous-apocrine differentiation could be best classified as a microcystic nail bed carcinoma arising from the follicular microcysts of the nail bed, with a limited differentiation toward the keratinization of the follicular isthmus.

Trichothiodystrophy: Photosensitive, TTD-P, TTD, Tay syndrome.

Although the term, "trichothiodystrophy" (TTD) refers to the hair anomalies in this group of patients, this is a heterogeneous, multisystem disease in which any or every organ in the body may be affected. Neuroectodermal derived tissues are particularly likely to be involved. This term was introduced by Price et alin 1980 to designate patients with sulfur-deficient brittle hair, which they recognized as a marker for this complex disease and designated it as a "neuroectodermal symptom complex". Patients with TTD have brittle hair and nails (associated with reduced content ofcysteine-rich matrix proteins), ichthyotic skin and physical and mental growth retardation. Ichthyosis is usually apparent at birth but much less so after the first few weeks of life. Other frequently associated features include ocular cataracts, infections and maternal complications related to pregnancy. Atrophy of subcutaneous fat may also be present. TTD occurs in a pattern of inheritance consistent with an autosomal recessive condition. The disease is extremely heterogeneous in severity and extent, with some patients showing no neurological deficiency. Others show severe, multisystem disease. Many patients die at a young age, most commonly due to infectious disease. TTD is part of a more broadly defined group of diseases identified as IBIDS (ichthyosis, brittle hair, impaired intelligence, decreased fertility and short stature). Photosensitive cases are also identified as PIBIDS (photosensitivity with IBIDS). Cases without manifest ichthyosis are also identified as PBIDS. These syndromes defy rigorous definition because of clinical variation between patients. The original two cases were described by Tay in oriental siblings, whose parents were first cousins; thus the disease is also known as Tay syndrome. The hairs in patients with TTD have a distinctive, diagnostically useful appearance on polarized light microscopy consisting of alternating light and dark bands known as the "tiger tail" anomaly. Diagnosis may be confirmed by sulfur content analysis ofhair shafts, which shows decreased sulfur and cysteine content. Approximately half of patients with TTD have photosensitivity, which correlates with a nudeotide excision repair (NER) defect. These patients are designated as having trichothiodystrophy-photosensitive (TTDP). Non-photosensitivepatients are designated as having trichothiodystrophy-nonphotosensitive (TTDN). Skin cancer is very rare in sun-sensitive TTD.

[Nail diseases in cosmetology]. / Choroby paznokci w praktyce kosmetologicznej.

Clinical symptoms attributed to the nail apparatus and observed in cosmetology include atrophic or hypertrophic lesions, pathologic nail coloration, abnormalities of the nail surface, and disorders of the nail plate and bed junction. These symptoms may reflect pathologic processes limited to the nail apparatus or may be the consequence of a dermal or systemic disease. Even though the etiology of nail lesions is variegated, diseases of the nails are simply classified as infectious or non-infectious. The aim of this work was to present the most common diseases of the nail apparatus encountered in cosmetology. Often, nail diseases worsen the quality of life of the patient. In addition, the variegated symptomatology demonstrates that nail lesions should be viewed in a wider perspective because they often are important signs of pathologic processes taking place in the organism of the patient.

A proposed subungual exostosis clinical classification and treatment plan.

Subungual exostosis is a slow-growing, benign outgrowth of normal bone under the nail that affects the nail unit. The most common location in the foot is the dorsal surface of the distal phalanx of the big toe. Clinically, it can appear in combination with a variety of nail disorders, masking the underlying bone condition, which is frequently unrecognized or misdiagnosed. A new classification system for these lesions is proposed on the basis of the clinical signs and symptoms present during examination and the associated disorders of the nail plate. Also, a therapeutic algorithm that describes surgical approaches to the different presentations of this disorder is presented.

Brittle nail syndrome: a pathogenesis-based approach with a proposed grading system.

Brittle nail syndrome is a heterogeneous abnormality, characterized by increased fragility of the nail plate. Brittle nails affect about 20% of the population and women are affected twice as frequently as men. The vast majority of patients experience brittle nails as a significant cosmetic problem and a substantial number indicate that these nail abnormalities are painful, impair daily activities, and may have a negative impact on occupational abilities. Pathogenic factors leading to brittle nails are factors that impair intercellular adhesion of the corneocytes of the nail plate or factors that cause a pathologic nail formation by involving the matrix. Clinical features of brittle nail syndrome are onychoschizia and onychorrhexis: the impairment of intercellular adhesive factors of the nail plate is expressed as onychoschizia, whereas the involvement of the nail matrix is expressed as onychorrhexis. Although impairment of life quality has not been evaluated for patients with brittle nail syndrome, the reduction of life quality in other nail problems has been studied and is evident. A proposed scoring system of key features of brittle nails is presented, and therapeutic approaches focussed on the pathogenic factors are discussed.