• Angela Hernández-Martin, Beatriz Aranegui, Ana Martin-Santiago, and Ignacio Garcia-Doval.
• Department of Dermatology, Hospital Infantil del Niño Jesús, Madrid, Spain. Electronic address: ahernandez_hnj@yahoo.es.
• J. Am. Acad. Dermatol. 2013 Oct 1; 69 (4): 544-549.e8.

BackgroundThe ichthyoses comprise a group of inherited disorders of keratinization. Because of the need for lifelong treatment, it is important that therapies are beneficial, safe, and well tolerated.ObjectivesWe sought to review the evidence on existing treatments for the congenital ichthyoses, excluding ichthyosis vulgaris.MethodWe undertook a systematic review using the methodology of the Cochrane Collaboration. Articles published in MEDLINE, EMBASE, and CENTRAL and registered clinical trials were screened. Randomized controlled trials involving patients with the inherited ichthyoses, either syndromic or nonsyndromic but excluding ichthyosis vulgaris, were considered.ResultsSix trials met the inclusion criteria. Topical treatments including 5% urea, 20% propylene glycol alone or in combination with 5% lactic acid, calcipotriol ointment, and liarozole 5% cream showed therapeutic benefit. Oral liarozole, a retinoic acid metabolism blocking agent, showed no advantage over oral acitretin.LimitationsMost studies were performed on a small sample of patients and lacked methodological and reporting quality. The small number of trials and the nearly constant positive results make publication bias likely. The absence of standardization of outcome measures precluded the comparison of studies.ConclusionsTopical treatments including emollients, calcipotriol ointment, and liarozole cream seem to have therapeutic benefit and a good safety profile, although the use of topical calcipotriol is limited by a maximum weekly dose of 100 g. The advantage of oral liarozole over acitretin is uncertain. Multicenter trials comparing oral and topical interventions and evaluation of long-term outcomes are needed.Copyright © 2013 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.

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