Iso-Kikuchi Syndrome: Report of 3 Pediatric Cases

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.

A 10-year-old Girl with a Melon Seed-like Nail on her Index Finger: A Quiz

“…Nail abnormalities have a wide variety of forms, including polyonychia, micronychia, anonychia, hemionychocryptosis, misalignment, and lunular deformity. Skeletal abnormalities can be seen on plain radiographs and commonly present as a Y-shaped bifurcation of the distal phalanges; however, they can also appear less frequently as brachydactyly, brachymetatarsi, and missing phalanges ( 6 ).…”