RAPK is usually inherited in an autosomal dominant pattern with high penetrance, although sporadic cases have been reported [50, 53].
In an exome sequencing study, ADAM10, a member of a disintegrin and metalloproteinase family, was found to be a causative gene for RAPK. The type of mutation in the ADAM10 gene could be a nonsense, missense, or splice site mutation .
The literature does not provide a consensus regarding the best therapeutic approach for RAPK. Several treatments have been tried with varied success rates.