[[Translated article]]Congenital and Hereditary Nail Disease. / Enfermedad ungueal congénita y hereditaria.

Nail disorders in newborns can show independently or as components of systemic illnesses or genodermatoses. The examination of these abnormalities is complex and sometimes challenging. However, familiarity with these disorders can significantly contribute to uncovering potential underlying conditions.This review includes the physiological nail changes seen within the first few months of life, such as Beau's lines, onychoschizia, koilonychia, congenital nail fold hypertrophy of the first digit, and onychocryptosis. This review also focuses on the most relevant congenital disorders reported and how to perform differential diagnosis. Finally, this review highlights those hereditary diseases in which nail involvement is crucial for diagnosis, such as nail-patella syndrome, congenital pachyonychia, or congenital dyskeratosis, among others.

Congenital and Hereditary Nail Disease. / Enfermedad ungueal congénita y hereditaria.

Nail disorders in newborns can show independently or as components of systemic illnesses or genodermatoses. The examination of these abnormalities is complex and sometimes challenging. However, familiarity with these disorders can significantly contribute to uncovering potential underlying conditions. This review includes the physiological nail changes seen within the first few months of life, such as Beau's lines, onychoschizia, koilonychia, congenital nail fold hypertrophy of the first digit, and onychocryptosis. This review also focuses on the most relevant congenital disorders reported and how to perform differential diagnosis. Finally, this review highlights those hereditary diseases in which nail involvement is crucial for diagnosis, such as nail-patella syndrome, congenital pachyonychia, or congenital dyskeratosis, among others.

Estudio de la correlación entre la onicodistrofia en los pies y el índice de masa corporal / Study of the correlation between onychodystrophy in the feet and the body mass index (BMI)

Fundamentos: Las alteraciones ungueales también se asocian a la obesidad. El objetivo de este trabajo fueevaluar la relación entre la onicodistrofia y el índice de masa corporal.Métodos: Se trata de un estudio observacional y transversal realizado en el Servicio de Angiología y CirugíaVascular del Hospital de la Santa Creu i Sant Pau de Barcelona, durante el período 2015-2017. La muestraconsta de 83 pacientes con una edad comprendida entre 40 y 84 años afectados de alteración venosa crónica,en una o en ambas extremidades inferiores. El análisis ungueal se realizó a través de iconografía yonicoscopia. El estado ponderal se estimó a partir del Índice de Masa Corporal (IMC) medido en kg/m2.Resultados: Dentro de la muestra de población estudiada, existe un ligero aumento de onicodistrofia cuantomayor es el índice de masa corporal. Sin embargo, este ligero aumento que se aprecia en la muestra no nospermite concluir, a un nivel de significación estadístico elevado, que también se producirá en la poblaciónobjeto de estudio.Conclusiones: Existe un ligero aumento de onicodistrofia cuanto mayor es el índice de masa corporal. (AU)

Background: Nail alterations are also associated with obesity. The objective was to evaluate the relationshipbetween onychodystrophy and the body mass index.Methods: This is an observational and transversal study conducted in the Angiology and Vascular SurgeryDepartment of the Hospital of the Santa Creu and Sant Pau of Barcelona, during the period 2015-2017. Thesample consists of 83 patients with an age between 40 and 84 years affected by chronic venous alteration, inone or both lower extremities. The nail analysis was performed through iconography and onicoscopy. Takinginto account the mass and height of the patients, their BMI was established.Results: In the population sample studied, there is a slight increase in onychodystrophy as the body massindex increases. However, this slight increase observed in the sample does not allow us to conclude, at a highlevel of statistical significance, that it will also occur in the population under study.Conclusions: There is a slight increase in onychodystrophy when the body mass index increases. (AU)

Anoniquia Congénita Asociada a Herencia Autosómica Dominante, Síndrome de Cooks

Resumen El síndrome de Cook fue descrito por primera vez por Cook y colaboradores en 1985. Este se caracteriza por una historia familiar de hipoplasia congénita de las uñas de las manos en los dígitos 1,2 y 3, ausencia de las uñas en los dígitos 4 y 5, braquidactilia del digito 5 de las manos y ausencia complete de las uñas de los pies. Además, puede existir una hipoplasia o ausencia de las falanges distales en los pies y las manos. La oficina de enfermedades raras del Instituto Nacional de Salud, considera este síndrome como una "enfermedad rara". Presentamos el caso de un recién nacido con anoniquia congénita en ambas manos y pies en el digito 2 asociado a hipoplasia ungueal en dígitos 1 y 3 respetando dígitos 4 y 5. La radiografía de los dedos no muestra anormalidades en las falanges. Este caso podría representar una variante del síndrome de Cook o una nueva enfermedad aun no descrita debido a la existencia de una historia familiar importante con similares deformidades en la madre, la abuela y la hermana.

Abstract Cooks syndrome, which was first reported by Cooks et al in 1985. It is characterized by family history of bilateral congenital nail hypoplasia of digits 1,2 and 3, with absence of nails in digits 4, 5, and brachydactyly of digit five of the hands and complete absence of all toenails. In addition, there is hypoplasia or absence of distal phalanges of the hands and feet. According to the Office of rare Diseases of the National Institutes of Health, this syndrome is considered as a "rare disease". We present a newborn child with a history of congenital anonychia in digit 2 in both hands and feet and nail hypoplasia in digits 1 and 3 sparing digits 4 and 5. Radiography of the fingers shows no abnormalities in the phalanges. This case could represent a variant of Cooks syndrome or a new disease not yet described because of the existence of an important family history with similar deformities in the mother, grandmother and sister.