Kayser-Fleischer rings

Kayser-Fleischer rings, sometimes shortened to K-F rings, are caused by copper deposition in the cornea and are a specific, clinical sign of Wilson disease.

They are usually brown or dark reddish in color. Early on they may need a slit lamp to be visible before they become visible to the naked eye. The ring can also be incomplete initially. Kayser-Fleischer rings are present in 95% of Wilson disease cases, including in nearly all cases with neurological manifestations 1,4. The density of the ring correlates well with the activity of the disease 1

The rings are not pathognomonic for Wilson disease.

Kayser-Fleischer rings are caused by direct copper deposition in the Descemet membrane of the cornea and are thought to be from epithelial cells absorbing copper from the aqueous humor 2

After commencement of copper chelating agents (e.g. penicillamine), the size of the rings usually diminish and may eventually vanish altogether 3.

They were initially described by German ophthalmologist Bernhard Kayser (1869-1954) 7 and German physician Bruno Fleischer (1848-1904) 7 in 1902 and 1903 respectively 5,6.

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Article information

rID: 60344
System: Head & Neck
Section: Signs
Synonyms or Alternate Spellings:
  • Kayser Fleischer rings
  • K-F rings

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