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Hereditary palmoplantar keratosis of the Gamborg Nielsen type: Clinical and ultrastructural characteristics of a new type of autosomal recessive palmoplantar keratosis by I. Kastl; I. Anton-Lamprecht; P. Gamborg Nielsen is a Medicine article available to read on EtoBox.

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A new kind of diffuse palmoplantar keratoderma with autosomal recessive inheritance and without associated symptoms was described in Norrbotten, Sweden by Gamborg Nielsen in 1985. Clinically, it ranges between the less severe dominant Unna-Thost type and the more severe recessive Meleda type, as it is milder than the latter. Skin biopsies of five patients from three different families with this new palmoplantar keratoderma, as well as five obligatory heterozygotes from one family, were investigated ultrastructurally in order to characterize this new entity and to differentiate it from the Meleda type. Several features are common to both autosomal recessive palmoplantar keratoses. They show a broadened granular layer, a transit region consisting of cells with a marginal envelope, and considerable hyperkeratosis. Morphologically, this transformation delay is less pronounced in the Gamborg Nielsen type than in the classical Meleda type. As is typical for ridged skin, both types of palmoplantar keratoses possess composite keratohyaline granules. In contrast to the normal appearance of keratohyaline granules in the Meleda type, the Gamborg Nielsen type also shows qualitative deviations

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Author

I. Kastl; I. Anton-Lamprecht; P. Gamborg Nielsen

Publisher

Springer-Verlag; Springer Verlag; Springer Science and Business Media LLC (ISSN 0340-3696)

Published

1990

Language

EN

Field

Medicine (Health Sciences)