The plaque-like or striate form of palmoplantar keratoderma, type Brunauer-Fuhs-Siemens, should be distinguished from the more common diffuse and punctate types of palmoplantar keratoderma. A family with typical clinical features of this autosomal dominantly inherited keratinisation disorder is presented. Treatment with systemic retinoids is effective. Lipidbiochemical analysis of plantar stratum corneum showed reduced amounts of total ceramides and an increase of free fatty acids. These results are possibly a secondary effect of the altered keratinisation. Linkage analysis could exclude both type I and type II keratin genes as candidate genes for this genodermatosis.
|Translated title of the contribution||Striate palmoplantar keratoderma. Clinical, lipidbiochemical and molecular investigations|
|Number of pages||6|
|Journal||H+G Zeitschrift fur Hautkrankheiten|
|Publication status||Published - 1 Jan 1995|