Author/Editor     Podrumac, B; Kansky, A; Prelog, I; Pejovnik-Pustinek, A
Title     Ichthyoses in Slovenian population: preliminary report
Type     članek
Source     Acta Dermatovenerol Alp Pannon Adriat
Vol. and No.     Letnik 6, št. 4
Publication year     1997
Volume     str. 145-9
Language     eng
Abstract     Introduction. Ichthyoses are a heterogeneous group of disorders, up to now no generally accepted classification exists. Additionally to the recessive x-linked ichthyosis (RXLI) in certain families of patients with lamellar ichthyosis (LI) the metabolic defect was elucidated. As known from previous studies the palmoplantar keratodermas and other genodermatoses are quite frequent in Slovenia. As there are no data on prevalence of ichthyosis in our country we decided to carry out a pilot study. Methods. Out- and in-patients' records were studied in the University Department of Dermatology in Ljubljana as well as in the dermatology departments of General Hospitals in Maribor and Celje. In Ljubljana data for a 20-year-period, in Maribor for a 10-year period and in Celje for an even shorter period were collected. Results. In Ljubljana 190 cases were recorded: 167 cases of ichthyosis vulgaris (the documentation did not allow a differentiation between the autosomal dominant ichthyosis and RXLI), 19 of non-bullous ichthyosiform erythroderma (NBIE) and 4 cases of LI. In Maribor there were 95 cases of ichthyosis vulgaris and 5 of NBIE. In Celje were registered 36 cases of autosomal dominant ichthyosis (ADI), 8 of RXLI, 4 of NBIE and 1 with neurological implication. Altogether 339 patients were recorded. Discussion. Although the material obtained is not suitable for an exact statistical analysis, the total of 339 cases of ichthyoses represents a rather high figure for a population of barely 2 million. The result includes only a minor number of affected family members. Conclusion. As the study is still in course, a substantially higher number of ichthyosis cases is to be expected.
Descriptors     ICHTHYOSIS
ICHTHYOSIS VULGARIS
ICHTHYOSIS, LAMELLAR
ICHTHYOSIS, X-LINKED
SLOVENIA