title: A systematic review of clinical trials of treatments for the congenital ichthyoses, excluding ichthyosis vulgaris.
abstract: BACKGROUND
  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.
  
  
  OBJECTIVE
  We sought to review the evidence on existing treatments for the congenital ichthyoses, excluding ichthyosis vulgaris.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Dermatology, Hospital Infantil del Niño Jesús, Madrid, Spain. Electronic address: ahernandez_hnj@yahoo.es.
authors.forename: Angela
authors.initials: A
authors.lastname: Hernández-Martin
authors.forename: Beatriz
authors.initials: B
authors.lastname: Aranegui
authors.forename: Ana
authors.initials: A
authors.lastname: Martin-Santiago
authors.forename: Ignacio
authors.initials: I
authors.lastname: Garcia-Doval
mesh_terms: D000284:Administration, Oral; D000287:Administration, Topical; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D016112:Ichthyosis Vulgaris; D017490:Ichthyosis, Lamellar / Q000175:diagnosis / Q000188:drug therapy*; D007641:Keratolytic Agents / Q000627:therapeutic use*; D008297:Male; D017060:Patient Satisfaction / Q000706:statistics & numerical data; D011379:Prognosis; D016032:Randomized Controlled Trials as Topic; D012176:Retinoids / Q000627:therapeutic use*; D018570:Risk Assessment; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D007641:Keratolytic Agents; D012176:Retinoids
keywords: ARCI; RCT; WHO; World Health Organization; X-linked ichthyosis; XLI; acitretin; autosomal recessive congenital ichthyosis; calcipotriol; congenital ichthyoses; emollients; liarozole; randomized controlled trial; systematic review