Iso-Kikuchi syndrome is a rare disorder characterized by congenital nail changes (anonychia or onychodysplasia) involving the index finger and sometimes accompanied by underlying bone alterations or involvement of other fingers or toes. Associations with other conditions are rare. The syndrome can be sporadic or hereditary. Familial cases are inherited in an autosomal dominant manner.

Diagnosis is largely clinical and familiarity with Iso-Kikuchi syndrome will favor early diagnosis, allowing patients and their families to receive the necessary information and avoiding unnecessary tests and treatments.

The article by Tirelly et al.1 in this issue of Actas Dermo-Sifiliográficas reminds us of the characteristics of this syndrome and describes 3 new pediatric cases.