<unk>  is inherited in an autosomal dominant manner, meaning an affected individual must inherit only one mutated allele from one parent. The protein, cryopyrin is coded for by the gene NLRP3, located at 1q44. The disease is frequent in Finland, and this population has a common mutation D21H accounting for all reported cases in this population. It has not been described in any other populations. However, the mutation was found in exome databases at a minor allele frequency (MAF) of 0.023% and in the Finnish and at an MAF of 0.0090% in aggregated non-Finnish European populations.
Keratoendotheliitis fugax hereditaria
Cutaneous lupus mucinosis
Poikiloderma vasculare atrophicans
Pemphigus vegetans
Orofacial granulomatosis
Acne medicamentosa
Hypotrichosis with juvenile macular dystrophy
Lycoperdonosis