Your chances of marrying a carrier of LI are 1 in 250.
If you marry any of those 249/250 that aren't carriers, your children will get 1 copy of the gene from you and a normal gene from your husband. They will not be affected, but they will all be carriers.
If you marry that 1/250 who is a carrier, you will pass on your copy, and your husband will give the normal copy to 50% and the LI copy to 50%. Half your kids will be carriers and half will be affected.
Your overall chances of having an affected kid are:
1/1 (you) x 1/250 (spouse) x 1/2 (2 possible outcomes). That's 1x250x2=500. 1/500 is considered extremely rare. It's about the same odds a 35yo person has of having a child with Down Syndrome.
Ichthyosis-en-Confetti Type 2
Husband, Nathan - 10, Elliot - 7, Oliver - 4, all affected.
I also have an unaffected daughter, age 8.
email: jennifer at confettiskin dot com
facebook - find me on "ichthyosis mommy spot" or "friends of ichthyosis"