Section Editor, Prof. Dr. Jan C. Simon, Leipzig
Nonsyndromic types of ichthyoses – an update
Version of Record online: 11 OCT 2013
© 2014 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.
JDDG: Journal der Deutschen Dermatologischen Gesellschaft
Volume 12, Issue 2, pages 109–121, February 2014
How to Cite
Traupe, H., Fischer, J. and Oji, V. (2014), Nonsyndromic types of ichthyoses – an update. JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 12: 109–121. doi: 10.1111/ddg.12229
The development of ichthyosis is now believed to result from genetic defects affecting the epidermal barrier along with a concomitant homeostatic repair response.
Among patients with two filaggrin mutations, about 30% do not have atopy; one filaggrin mutation is sufficient for predisposing patients to atopic eczema or allergic rhinitis.
Newborns with harlequin ichthyosis or who are born as a collodion baby are considered a “dermatological emergency” and should be transferred to a neonatal intensive care unit.
Patients with ARCI or keratinopathic ichthyosis often have significant vitamin D deficiency.
Long daily baths and mechanical keratolysis are essential for patients with severe ichthyosis.
Patients should be encouraged to contact and join a self-help organization.
- Issue online: 4 FEB 2014
- Version of Record online: 11 OCT 2013
- Manuscript Accepted: 29 AUG 2013
- Manuscript Received: 5 AUG 2013
- 2Ichthyoses: Clinical, biochemical, pathogenic and diagnostic assessment. Karger, Basel, 2010., , , .
- 4Filaggrin haploinsufficiency is highly penetrant and is associated with increased severity of eczema: further delineation of the skin phenotype in a prospective epidemiological study of 792 school children. Br J Dermatol 2009; 61: 884–9., , et al.