S1 guidelines for the diagnosis and treatment of ichthyoses – update

  title={S1 guidelines for the diagnosis and treatment of ichthyoses – update},
  author={Vinzenz Oji and Marie-Luise Preil and Barbara Kleinow and G Wehr and Judith Fischer and Hans Christian Hennies and Ingrid Hausser and Dirk Breitkreutz and Karin Aufenvenne and Karola Maria Stieler and Illiana Tantcheva-Po{\'o}r and Stefan Weidinger and Steffen Emmert and H Hamm and Ana Mar{\'i}a Perusqu{\'i}a-Ortiz and Irina Zaraeva and Anja Diem and Kathrin A. Giehl and Regina F{\"o}lster-Holst and Kirstin Kiekbusch and Peter H. H{\"o}ger and Hagen Ott and Heiko Traupe},
  journal={JDDG: Journal der Deutschen Dermatologischen Gesellschaft},
Ichthyoses are a group of rare genetic skin disorders that pose numerous clinical challenges, in particular with respect to the correct diagnosis and appropriate management. The present update of the German ichthyosis guidelines addresses recent diagnostic advances that have resulted in the Sorèze consensus classification. In this context, we provide an updated diagnostic algorithm, taking into account clinical features as well as the molecular genetic basis of these disorders. Moreover, we… 
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  • 2019
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  • 2013
A recent consensus conference revised the nomenclature and proposed a new classification that distinguishes 36 different types of ichthyosis and 10 related clinical entities (for example, CHILD-syndrome).
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The diagnostic steps are based on clinical data, analyses such as the steroid sulfatase activity test, skin biopsies, and genetic results, and the dramatic increase in knowledge about the pathophysiology of these conditions has not led to a curative therapy so far.
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Considering that patients with ichthyosis are potential mega-users of topical therapy, with an estimated lifetime consumption of approximately one tonne cream per capita, surprisingly few controlled trials of the various treatments have been performed.
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Revised nomenclature and classification of inherited ichthyoses: results of the First Ichthyosis Consensus Conference in Sorèze 2009.
An international consensus is achieved for the classification of inherited ichthyosis that should be useful for all clinicians and can serve as reference point for future research.
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Ichthyoses are genetically determined Mendelian disorders of cornification (MEDOC) that are characterized by universal scaling. Today we distinguish between non‐syndromic and syndromic forms.