caa a22 4500 465773583 CHVBK 20180323111935.0 cr unu---uuuuu 170327e19900601xx s 000 0 eng 10.1007/BF00371641 doi (NATIONALLICENCE)springer-10.1007/BF00371641 Altered keratin expression in ichthyosis hystrix Curth-Macklin [Elektronische Daten] A light and electron microscopic study [K. Niemi, I. Virtanen, L. Kanerva, M. Muttilainen] Summary: The pathogenesis of a rare form of the ichthyotic diseases, ichthyosis hystrix Curth-Macklin, was investigated by immunohistochemistry and electron microscopy. Monoclonal antibodies (Mabs) against keratins expressed in normal basal cells (PKK2 and KA1), Mabs against keratins only present in normal fetal skin (PKK1), and Mabs against keratins 1, 2, 10, and 11 (KA5 and K8.60) were used. The Mabs reacting with normal basal cells showed an increased reaction with many cell layers. The Mab PKK1 distinctly reacted with the basal cell layer, suggesting an expression of fetal keratins. Electron microscopic study of both normal-looking and involved skin revealed the keratinization disorder characterized by tonofilament shells, perinuclear vacuoles, and binuclear keratinocytes. The results suggest that there is no prematurity of keratinization, but rather a pathological expression of specific keratin genes leading to expression of fetal keratins in this form of ichthyosis hystrix. Springer-Verlag, 1990 Genodermatoses nationallicence Keratinization disorders nationallicence Fetal cytokeratins nationallicence Immunohistochemistry nationallicence Electron microscopy nationallicence Niemi K. Department of Dermatology, Helsinki University Central Hospital, Snellmaninkatu 14, SF-00170, Helsinki, Finland aut Virtanen I. Department of Anatomy, University of Helsinki, Finland aut Kanerva L. Institute of Occupational Health, Helsinki, Finland aut Muttilainen M. Jorvi Hospital, Espoo, Finland aut Archives of Dermatological Research Springer-Verlag 282/4(1990-06-01), 227-233 0340-3696 282:4<227 1990 282 403 https://doi.org/10.1007/BF00371641 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF00371641 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Niemi K. Department of Dermatology, Helsinki University Central Hospital, Snellmaninkatu 14, SF-00170, Helsinki, Finland aut NATIONALLICENCE 700 1- Virtanen I. Department of Anatomy, University of Helsinki, Finland aut NATIONALLICENCE 700 1- Kanerva L. Institute of Occupational Health, Helsinki, Finland aut NATIONALLICENCE 700 1- Muttilainen M. Jorvi Hospital, Espoo, Finland aut NATIONALLICENCE 773 0- Archives of Dermatological Research Springer-Verlag 282/4(1990-06-01), 227-233 0340-3696 282:4<227 1990 282 403 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer