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Genetic and Rare Diseases Information Center (GARD)

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Kindler syndrome

Other Names for this Disease
  • Bullous acrokeratotic poikiloderma of kindler and weary
  • Congenital bullous poikiloderma
  • Poikiloderma of Kindler
  • Poikiloderma, congenital, with bullae, weary type
  • Poikiloderma, hereditary acrokeratotic
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Your Question

My mother had Kindler Syndrome. I now have two daughters of my own. It never even crossed my mind during my pregnancies that I might be passing along this gene or give birth to a child with Kindler Syndrome.  In general, what is the likelihood of me having a child with this disease?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is the likelihood of having a child with Kindler syndrome?

Kindler syndrome is inherited in an autosomal recessive pattern.[1]  This means that both parents must be a carrier of a mutation in the FERMT1 gene for there to be a chance that their child could have Kindler syndrome.  Because Kindler syndrome is so rare[1], it is very unlikely that both members of a couple are carriers.  However, if both parents are carriers, there is a 25% chance of the child being affected with Kindler syndrome, a 50% chance of the child being a carrier, and a 25% chance of the child being unaffected and not a carrier.
Last updated: 9/23/2013