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Journal of the American Academy of Dermatology
Sous presse. Epreuves corrigées par l'auteur. Disponible en ligne depuis le mercredi 17 juillet 2013
Doi : 10.1016/j.jaad.2013.05.017
accepted : 17 May 2013
A systematic review of clinical trials of treatments for the congenital ichthyoses, excluding ichthyosis vulgaris

Angela Hernández-Martin, MD a, , Beatriz Aranegui, MD b, Ana Martin-Santiago, MD c, Ignacio Garcia-Doval, PhD b
a Department of Dermatology, Hospital Infantil del Niño Jesús, Madrid, Spain 
b Research Unit, Fundación Academia Española de Dermatología y Venereología, Madrid, Spain 
c Department of Dermatology, Hospital Universitario Son Dureta, Palma de Mallorca, Spain 

Reprint requests: Angela Hernández-Martin, MD, Research Unit, Fundación Academia Española de Dermatología y Venereología, c/ Ferraz, 100 1°D, 28008 Madrid, Spain.

The 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.


We sought to review the evidence on existing treatments for the congenital ichthyoses, excluding ichthyosis vulgaris.


We 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.


Six 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.


Most 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.


Topical 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.

The full text of this article is available in PDF format.

Key words : acitretin, calcipotriol, congenital ichthyoses, emollients, liarozole, randomized controlled trial, systematic review

Abbreviations used : ARCI, RCT, WHO, XLI

 This systematic review has been supported by the Research Unit of the Fundación Academia Española de Dermatología y Venereología (Spanish Academy of Dermatology).
 Conflicts of interest: None declared.

© 2013  American Academy of Dermatology, Inc.@@#104156@@
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