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Publication Details
AFRICAN RESEARCH NEXUS
SHINING A SPOTLIGHT ON AFRICAN RESEARCH
medicine
Revised nomenclature and classification of inherited ichthyoses: Results of the First Ichthyosis Consensus Conference in Sorze 2009
Journal of the American Academy of Dermatology, Volume 63, No. 4, Year 2010
Notification
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Description
Background: Inherited ichthyoses belong to a large, clinically and etiologically heterogeneous group of mendelian disorders of cornification, typically involving the entire integument. Over the recent years, much progress has been made defining their molecular causes. However, there is no internationally accepted classification and terminology. Objective: We sought to establish a consensus for the nomenclature and classification of inherited ichthyoses. Methods: The classification project started at the First World Conference on Ichthyosis in 2007. A large international network of expert clinicians, skin pathologists, and geneticists entertained an interactive dialogue over 2 years, eventually leading to the First Ichthyosis Consensus Conference held in Sorze, France, on January 23 and 24, 2009, where subcommittees on different issues proposed terminology that was debated until consensus was reached. Results: It was agreed that currently the nosology should remain clinically based. "Syndromic" versus "nonsyndromic" forms provide a useful major subdivision. Several clinical terms and controversial disease names have been redefined: eg, the group caused by keratin mutations is referred to by the umbrella term, "keratinopathic ichthyosis"-under which are included epidermolytic ichthyosis, superficial epidermolytic ichthyosis, and ichthyosis Curth-Macklin. "Autosomal recessive congenital ichthyosis" is proposed as an umbrella term for the harlequin ichthyosis, lamellar ichthyosis, and the congenital ichthyosiform erythroderma group. Limitations: As more becomes known about these diseases in the future, modifications will be needed. Conclusion: We have achieved an international consensus for the classification of inherited ichthyosis that should be useful for all clinicians and can serve as reference point for future research. © 2009 by the American Academy of Dermatology, Inc.
Authors & Co-Authors
Oji, Vinzenz
Germany, Munster
Universitätsklinikum Münster
Tadini, Gianluca
Italy, Milan
Fondazione Irccs Ca' Granda Ospedale Maggiore Policlinico
Akiyama, Masashi
Japan, Sapporo
Graduate School of Medicine
Blanchet-Bardon, Claudine
France, Paris
Hôpital Saint-louis
Bodemer, Christine
France, Paris
Ap-hp Assistance Publique - Hopitaux de Paris
Bourrat, Emmanuelle
France, Paris
Hôpital Saint-louis
Coudiere, Philippe
France, Paris
Groupe Pierre Fabre
Digiovanna, John J.
United States, Providence
The Warren Alpert Medical School
Elias, Peter
United States, San Francisco
San Francisco va Health Care System
Fischer, Judith
France, Evry
Centre National de Recherche en Génomique Humaine
Fleckman, Philip
United States, Seattle
University of Washington
Gina, Michal
Switzerland, Lausanne
Centre Hospitalier Universitaire Vaudois
Harper, John I.
United Kingdom, London
Great Ormond Street Hospital for Children Nhs Foundation Trust
Hashimoto, Takashi
Japan, Kurume
Kurume University School of Medicine
Hausser, Ingrid
Germany, Heidelberg
Universitätsklinikum Heidelberg
Hennies, Hans C.
Germany, Koln
Medizinische Fakultät
Hohl, Daniel M.
Switzerland, Lausanne
Centre Hospitalier Universitaire Vaudois
Hovnanian, Alain A.
France, Paris
Hôpital Necker Enfants Malades
France, Paris
Inserm
Ishida-Yamamoto, Akemi
Japan, Asahikawa
Asahikawa Medical University
Jacyk, Witold Kamil
South Africa, Pretoria
University of Pretoria
Leachman, Sancy
United States, Salt Lake City
University of Utah Health Sciences
Leigh, Irene May M.O.P.
United Kingdom, London
Barts and the London School of Medicine and Dentistry
Mazereeuw-Hautier, Juliette H.
France, Toulouse
Hôpital Purpan
Milstone, Leonard M.
United States, New Haven
Yale University
Morice-Picard, Fanny
France, Bordeaux
Hopital Saint-andre
Paller, Amy S.
United States, Chicago
Northwestern University Feinberg School of Medicine
Richard, Gabriele
United States, Gaithersburg
Genedx, Inc.
Schmuth, Matthias
United States, San Francisco
University of California, San Francisco
Austria, Innsbruck
Medizinische Universitat Innsbruck
Shimizu, Hiroshi
Japan, Sapporo
Graduate School of Medicine
Sprecher, Eli
Israel, Tel Aviv-yafo
Tel Aviv Sourasky Medical Center
Van Steensel, Maurice
Netherlands, Maastricht
Faculty of Health, Medicine and Life Sciences
Taïeb, Alain B.
France, Bordeaux
Hopital Saint-andre
Toro, Jorge R.
United States, Rockville
National Cancer Institute Nci
Vabres, Pierre
France, Dijon
Université de Bourgogne
Vahlquist, Anders
Sweden, Uppsala
Uppsala Universitet
Williams, Mary
United States, San Francisco
University of California, San Francisco
Traupe, Heiko
Germany, Munster
Universitätsklinikum Münster
Statistics
Citations: 666
Authors: 37
Affiliations: 33
Identifiers
Doi:
10.1016/j.jaad.2009.11.020
ISSN:
01909622