“…94 Diseases relatively new in the list of ichthyoses are loricrin keratoderma, also referred to as Camisa variant of Vohwinkel keratoderma (Fig 4, C ), [95][96][97] the cerebral dysgenesiseneuropathyeichthyosisePPK syndrome, 98 the arthrogryposiserenal dysfunctionecholestasis syndrome, 99-101 the mental retardationeenteropathye deafnesseneuropathyeichthyosisekeratodermia syndrome, 102 the ichthyosisehypotrichosisesclerosing cholangitis syndrome (also known as neonatal ichthyosis sclerosing cholangitis syndrome), 103-105 the ichthyosis hypotrichosis syndrome (Fig 5, I ) 106 and its allelic variant congenital ichthyosisefollicular atrophodermaehypotrichosisehypohidrosis syndrome, 107,108 and keratosis lineariseichthyosisecongenital sclerosing keratoderma (Fig 4, F ). 109,110 Erythrokeratodermia variabilis (EKV), [111][112][113] which is characterized by migratory erythematous patches and more fixed, symmetric hyperkeratotic plaques often with palmoplantar involvement (Fig 4, B), is genetically heterogeneous and can in 50% to 65% of cases 114 be caused by mutations in GJB3 coding for the gap junction protein connexin 31, 115 or GJB4 coding for connexin 30.3. 116 Whether Acral PSS, Acral peeling skin syndrome; AD, autosomal dominant; AR, autosomal recessive; CE, cornified cell envelope; CIE, congenital ichthyosiform erythroderma; EKV, erythrokeratodermia variabilis; IE, ichthyosiform erythroderma; KG, keratohyaline granules; KLICK, keratosis lineariseichthyosis congenitaekeratoderma; LB, lamellar body; LK, loricrin keratoderma; PPK, palmoplantar keratoderma; PSD, peeling skin disease; SC, stratum corneum; SG, stratum granulosum.…”