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Hereditary palmoplantar keratoderma, type papulosa, in Croatia
A Stanimirović1, A Kansky, A Basta-Juzbasić
1Department of Dermatology and Venereology, Medical School of Zagreb University, Croatia.
Journal of the American Academy of Dermatology
|September 1, 1993
Summary
Hereditary palmoplantar keratoderma (HPPK), type papulosa, is a rare skin condition. This study found its prevalence in Croatia to be 1.17 per 100,000 inhabitants, suggesting it is a distinct disease entity.
Area of Science:
- Dermatology
- Genetics
- Epidemiology
Background:
- Hereditary palmoplantar keratoderma (HPPK), type papulosa, is a rare condition with inconsistent epidemiologic data.
- Previous population studies on HPPK, type papulosa, are lacking.
Purpose of the Study:
- To conduct a large population study on the prevalence of HPPK, type papulosa, in Croatia.
- To establish the epidemiologic characteristics of this rare dermatologic disorder.
Main Methods:
- Data collection from dermatology departments across Croatia.
- Examination of 14 affected patients and their relatives.
- Histopathologic studies performed on 11 patients.
Main Results:
- Identified 55 patients with HPPK, type papulosa, yielding a prevalence of 1.17 per 100,000 inhabitants.
- All patients belonged to 20 distinct families, with autosomal dominant inheritance confirmed in 13.
- Affected individuals presented with palmar, plantar, and finger lesions; nail thickening was noted in four.
Conclusions:
- HPPK, type papulosa, is rare in Croatia, with a prevalence approximately four times lower than the Unna-Thost type.
- The findings support HPPK, type papulosa, as a distinct clinical and genetic entity.
- Further research into the specific genetic and clinical features of HPPK, type papulosa, is warranted.