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Hereditary epidermolytic palmoplantar keratoderma (Vo @?rner type) in a family with Ehlers-Danlos syndrome
- Source :
- Journal of the American Academy of Dermatology; May 1998, Vol. 38 Issue: 5 p825-830, 6p
- Publication Year :
- 1998
-
Abstract
- We describe a kindred in whom epidermolytic palmoplantar keratoderma occurred in association with Ehlers-Danlos syndrome type III (benign hypermobility syndrome). This kindred consisted of 27 members of four generations, 14 of whom had palmoplantar keratoderma (PPK). Of those who had palmoplantar keratoderma, 6 had Ehlers-Danlos type III (EDS III). The proband presented with diffuse, symmetrical hyperkeratotic plaques that were yellow and sharply demarcated, covering the entire palms and soles, in addition to marked large and small joint flexibility and skin hyperextensibility. A biopsy specimen from the palm revealed features of epidermolytic hyperkeratosis with acanthosis. To our knowledge, this is the first report of PPK in a family with Ehlers-Danlos syndrome. Linkage analysis of these two clinical traits showed that the genes responsible for PPK and EDS III are not closely linked, and therefore are not immediately adjacent. However, linkage at greater genetic distances could not be excluded. (J Am Acad Dermatol 1998;38:825-30.)
Details
- Language :
- English
- ISSN :
- 01909622 and 10976787
- Volume :
- 38
- Issue :
- 5
- Database :
- Supplemental Index
- Journal :
- Journal of the American Academy of Dermatology
- Publication Type :
- Periodical
- Accession number :
- ejs9924359
- Full Text :
- https://doi.org/10.1016/S0190-9622(98)70467-0