A systematic review of clinical trials of treatments for the congenital ichthyoses, excluding ichthyosis vulgaris.

[lamellar ichthyosis]

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 .

Diseases
Validation

Diseases presenting "small number" symptom

adrenal incidentaloma

allergic bronchopulmonary aspergillosis

aromatase deficiency

cadasil

carcinoma of the gallbladder

cohen syndrome

cushing syndrome

dedifferentiated liposarcoma

dentinogenesis imperfecta

dracunculiasis

erdheim-chester disease

focal myositis

gm1 gangliosidosis

hodgkin lymphoma, classical

homocystinuria without methylmalonic aciduria

junctional epidermolysis bullosa

kabuki syndrome

kindler syndrome

lamellar ichthyosis

legionellosis

liposarcoma

locked-in syndrome

malignant atrophic papulosis

oculocutaneous albinism

oligodontia

omenn syndrome

pleomorphic liposarcoma

pyruvate dehydrogenase deficiency

severe combined immunodeficiency

thoracic outlet syndrome

triple a syndrome

waldenström macroglobulinemia

well-differentiated liposarcoma

wiskott-aldrich syndrome

wolf-hirschhorn syndrome

zellweger syndrome

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