Palmoplantar keratoderma with an unusual composition of stratum corneum and serum sterol derivatives: a new entity?

Abstract

Little is known about the aetiology and pathogenesis of the different types of inherited and acquired palmoplantar keratodermas. We describe a condition of painful palmoplantar keratoderma with an altered stratum corneum lipid pattern which may be responsible for the excessive cornification. Plantar stratum corneum lipids were analysed by quantitative thin-layer chromatography. Serum lipids, and the activities and gene loci of the enzymes serum steroid sulphatase and arylsulphatase C were also determined. Examination revealed that both the stratum corneum and the serum cholesterol sulphate (CS) content were significantly elevated in comparison with the stratum corneum cholesterol ester content. The cholesterol content was unchanged compared with controls. Serum activities of steroid sulphatase and arylsulphatase C were decreased, but not to the extent found in recessive X-linked ichthyosis. Their gene loci did not show any deletions. This unique distribution of stratum corneum sterol derivatives, reflected by the elevated serum CS concentration, may contribute to the altered structural and functional properties of intercellular lipid lamellae within the stratum corneum of this type of keratoderma.

Cholesterol sulfate induces changes in human erythrocyte thermostability

“…Its ability to inhibit tumour promotion mediated by the rl isoform of protein kinase C additionally proves the biological importance of this compound [26]. Furthermore, CS has been the subject of extensive investigations due to its connections with several serious diseases, such as recessive X-linked ichthyosis [10,27], liver cirrhosis [28], familial hypercholesterolemia [28], homozygous sickle-cell anaemia [29], diabetes mellitus [30] and palmoplanar keratoderma [31 ] where abnormal CS levels have been reported.…”