Kongenitale Onychodysplasie (Iso-Kikuchi-Syndrom)

Abstract

A 1-year-old girl shows changes of the toenails since birth. There are no associated anomalies typical for genodermatoses, nor is there a family history of this disorder. Physical examination shows missing or incomplete toenails. In addition a shortening of the second toe on both sides and the third toe on the left side is found. The second and third toe on the left side shows incomplete syndactylia. The fingers and fingernails are all normal. Isolated congenital onychodysplasia of the toenails has not been previously reported.

Congenital isolated Iso–Kikuchi syndrome in a newborn

“…Since these first observations, clinical criteria have been expanded to include a number of additional associated conditions derived from small series, case reports, and retrospective reviews over the next 30 years. CO clinical criteria are the following: unilateral or bilateral hypoplasia of the index fingernails and/or other fingers including toenails 3 (up to total anonychia of hands and feet); radiographic abnormalities of the distal bony phalanx of the affected fingers; congenital occurrence, which can be both sporadic or hereditary 4 , 5 . …”

“…unilateral or bilateral hypoplasia of the index fingernails and/or other fingers including toenails 3 (up to total anonychia of hands and feet);…”

Congenital onychodysplasia of the ring finger presenting as a bifid nail

“…However, three cases have been reported with associated ear abnormalities, congruent with our patient. 1,5 Treatment of congenital onychodysplasia is optional and usually dictated by symptoms or cosmetic concerns. Due to the pain associated with our patient's dystrophic nail, urea cream has been used to soften the nail; however, if unresponsive, partial matricectomy of the curved portion of the nail will be considered.…”