Vortex keratopathy
 

This is a common physical signs in the examination but unlikely to form the only signs in the examination. Look for  another pathology. There are corneal deposits (in the basal layer of the epithelium) distributed in whorl's like pattern (cornea verticillata). Look for signs of : amiodarone intake (for example photosensitivity of the skin, irregular pulse rate and there may be branch retinal  or central retinal artery occlusion from emboli) indomethacin intake (look for signs of rheumatoid arthritis) Fabry's disease (renal failure with arteriovenous fistula, angiokeratomas, dilated and tortuous  conjucntival vessels with or without aneurysms). .

Questions:

1. What is Fabry's disease?

Answer
It is an X-linked disorder due to a deficiency of alpha-galactosidase. It causes depositon of glycosphingolipid in the body tissue. Renal involvement can cause renal failure. Skin lesion termed angiokeratoma corporis discusum consists of clusters of superificial cutaneous dark-red angiokeratomas.
Apart from vortex keratopathy, there may be tortuosity and aneurysmal dilatations of the conjunctival and retinal vessels.

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