Oji, V., Preil, M.L., Kleinow, B., Wehr, G., Fischer, J., Hennies, Hans C., Hausser, I., Breitkreutz, D., Aufenvenne, K., Stieler, K., Tantcheva- Poór, I., Weidinger, S., Emmert, S., Hamm, H., Perusquia-Ortiz, A.M., Zaraeva, I., Diem, A., Giehl, K., Fölster-Holst, R., Kiekbusch, K., Höger, P., Ott, H. and Traupe, H. (2017) S1 guidelines for the diagnosis and treatment of ichthyoses – update. Journal der Deutschen Dermatologischen Gesellschaft, 15 (10). pp. 1053-1065. ISSN 1610-0379
Abstract

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 highlight
current therapeutic approaches such as psychosocial support, balneotherapy, mechanical
scale removal, topical therapy, and systemic retinoid therapy. General aspects
such as the indication for physical therapy, ergotherapy, or genetic counseling are
also discussed. The present update was consented by an interdisciplinary consensus
conference that included dermatologists, pediatricians, human geneticists, and natural
scientists as well as representatives of the German patient support organization
Selbsthilfe Ichthyose e. V.

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