The Chanarin Dorfman syndrome (CDS) is a rare autosomal recessive disorder characterized by an inborn error of nonlysosomal neutral lipid metabolism. Clinical expression is variable involving multiple organs.1 Cutaneous manifestations, including a nonbullous congenital ichthyosiform erythroderma, are the most common symptoms. Fewer than 50 cases have been reported to date, and most patients are from Middle Eastern countries or France.
Schleinitz N, Fischer J, Sanchez A, Veit V, Harle J, Pelissier J. Two New Mutations of the ABHD5 Gene in a New Adult Case of Chanarin Dorfman Syndrome: An Uncommon Lipid Storage Disease. Arch Dermatol. 2005;141(6):798-800. doi:10.1001/archderm.141.6.798