Actas Dermo-Sifiliográficas (English Edition) Actas Dermo-Sifiliográficas (English Edition)
e- Case Report
Iso-Kikuchi Syndrome: Report of 3 Pediatric Cases
Síndrome de Iso-Kikuchi: 3 casos en la edad pediátrica
L.L. Tirelli, , P.C. Luna, R. Cano, J.P. Giraldo, M. Larralde
Servicio de Dermatología, Hospital Alemán, Ciudad Autónoma de Buenos Aires, Argentina
Abstract

Iso-Kikuchi syndrome, or congenital onychodysplasia of the index finger, is an uncommon condition characterized by total anonychia or dysplasia of the nail of the index finger. It is occasionally accompanied by underlying bone abnormalities and is rarely associated with other conditions. Although various hypotheses have been put forward to explain the pathophysiology of the syndrome, its etiology remains unknown.

We report the cases of 3 pediatric patients (2 boys and 1 girl) with nail changes and bone abnormalities consistent with Iso-Kikuchi syndrome. We highlight the importance of recognizing this entity early to avoid the need for additional tests and unnecessary treatment.

Resumen

El síndrome de Iso-Kikuchi, onicodisplasia congénita del dedo índice, es una entidad poco frecuente caracterizada por la anoniquia total o displasia de la uña del dedo índice, acompañado, en algunas ocasiones, de alteraciones óseas subyacentes, por lo general, en ausencia de otras anomalías. Si bien se han planteado distintas hipótesis fisiopatogénicas, la etiología sigue siendo desconocida.

Describimos los casos de 3 pacientes pediátricos, 2 varones y una niña, con alteraciones ungueales y óseas compatibles con el síndrome de Iso-Kikuchi. Destacamos la importancia de reconocer esta entidad tempranamente para evitar la realización de estudios complementarios y terapéuticas innecesarias.

Keywords
Iso-Kikuchi, Anonychia, Nail, Index finger, Phalanx
Palabras clave
Iso-Kikuchi, Anoniquia, Uña, Dedo índice, Falange

Article

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