Author/Editor		Miljković, Jovan; Kansky, Aleksej; Arzenšek, Jože; Vidmar, Gaj; Kokol, Peter
Title		Difuzne palmoplantarne keratodermije v Sloveniji
Type		članek
Source		In: Miljković J, editor. 2. dermatološki dnevi. Zbornik predavanj Strokovno izpopolnjevanje iz dermatologije z mednarodno udeležbo; 2004 nov 5-7; Maribor. Maribor: Splošna bolnišnica Maribor,
Publication year		2004
Volume		str. 27-40
Language		slo
Abstract		During our studies on hereditary disorders of keratinisation the obsernation was made that in certain disorders some additional factors play a role in expression of symptoms beside the autosomal dominant gene. In an attempt to clarify this problem the epidemiological approach was used, diffuse palmoplantar keratoderma (DPPK) was studied. Data on patients for last 30 years (1973-2003) were collected from hospital records, by routine clinical work as mell as by visiting patients at their homes. The data were used for compilation of pedigrees. 170 patients with diffuse HPPK were registered, 34 of them mere isolated cases. The prenalence is 8,3 : 100000. The ratio affected/unaffected was 0,485: the involved patients ratio differs high significant than 50% (exact binom test: p < 0,00001). The segregation ratio shomed autosomal dominant mode of inheritance (p = 0,37406). Almost all families origina ed from remote mountanious areas in Slovenia, where people were living in isolation for centuries. Prevalence in these areas differs high significant than prevalence in total Slovenian population (hi-quadrat test: p < 0,00001). There were less affected persons among the younger generations and theirsymptoms mere milder. It is estimated that the main factors contributing to the expression of symptoms DPPK mere consanguinity due to the isolation and agricultural activities
Descriptors		KERATODERMA, PALMOPLANTAR, DIFFUSE CONSANGUINITY SLOVENIA