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Congenital onychodysplasia - Nail and Distal Digit
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Congenital onychodysplasia - Nail and Distal Digit

Contributors: Bertrand Richert MD, Robert Baran MD
Other Resources UpToDate PubMed

Synopsis

Congenital onychodysplasia of the index finger, also known as Iso-Kikuchi syndrome, is a disorder characterized by a variety of nail deformities of the index fingers and bone abnormalities. Nail abnormalities include micronychia, "rolled" micronychia, polyonychia, hemionychogryphosis, malalignment, anonychia, and onychoheterotopia. The most common bone abnormality is a Y-shaped bifurcation of the distal phalanx, which may occur under both normal and abnormal nails. The defects occur most commonly on the radial side of the index fingers.

Codes

ICD10CM:
Q79.8 – Other congenital malformations of musculoskeletal system

SNOMEDCT:
403281007 – Congenital onychodysplasia of index fingers

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Congenital malalignment of the hallux and the second toenail
  • Polyonychia in syndactyly
  • Central micronychia
  • Polyonychia in congenital skin anomaly
  • Onychoheterotopia

Best Tests

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Management Pearls

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Therapy

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References

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Last Updated:01/19/2015
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Congenital onychodysplasia - Nail and Distal Digit
Congenital onychodysplasia : Absent nail plate, Fingernails, Micronychia, Present at birth, Short nail
Clinical image of Congenital onychodysplasia
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