treatment of the recessive nonbullous congenital ichthyosis by the epigallocatechine cutaneous

Conditions: Lamellar Ichthyosis

Summary: lamellar ichthyosis (il) is a rare autosomal recessive genodermatosis with a defect of keratinization of the skin which results in a severe generalized cutaneous xerosis with dark brown big scales, an ectropion, an eclabion, an alopecia and a palmo-plantar keratodermia. they are due to mutations of the gene tgm1 coding for the transglutaminase keratinocyte 1 (tg1) in 1/3 of the cases. other genes were recently identified, abca12 coding for the triphosphate-binding adenosine cassette a12 and flj39501 which codes for a protein of the cytochrome p450 ( cyp4f2). no etiological treatment is available. symptomatic treatment consists on twice application of emollients and keratolytic ointments which decrease the dryness of the skin and reduce scales. oral isotretinoin is usually partially effective but is only suspensive and has numerous side effects. recent studies showed that the epigallocatechin-3-gallate (polyphenon e®), extracted from green tea increases the differentiation of the normal human keratinocytes, as showedb by the increase of the involucrine, tg1 and caspase-14 genes expression. the main objective of this pilot study is to estimate the action and the tolerance of a daily application of topical polyphénon e 10% ® to improve the desquamation and the cutaneous roughness of patients with lamellar ichthyosis, after 4 weeks of treatment. the secondary objectives * to estimate the duration of remission obtained after the treatment * to estimate the action of cutaneous veregen® to improve the palmar and plantar involvement. * to estimate the action of cutaneous veregen on the pruritus * and to estimate the global level of acceptability by the patient of the veregen 10 %


Eligibility criteria:
inclusion criteria: * patients of both sexes of at least 8 years and less than 65 years. * patients with a clinical diagnosis of li * patients having at once a score of roughness and a desquamation of intensity moderated in severe (at least 2) on every side of the body, * patients and\\or relatives / representatives of the parental authority in measure to understandand to follow the procedures of the study * consent of patient and\\or parents / representatives of the parental authority * patient member to the social security exclusion criteria: * patient of less than 8 years * pregnant, breast-feeding women or old enough to procreate without reliable medical contraception, * women with a positive pregnancy test, * transaminases \> twice the normal. * patients with congenital ichthyosis others than li, * patients with a erythrodermic composent, * patients affected by li of the light gravity (score \< 2 for the desquamation or the roughness) on at least a side of the body, * patients with secondary infection , * patients with known allergy of to one of the ingredients contained in the tested product, * patients with specific topical treatment (for example analogues of vitamin a, vitamin d similar), * patients with topical keratolytic treatment (for example the urea, the hydroxy-acids) in 7 days before the beginning of the clinical trial * patients and\\or relatives / representatives of the parental authority unable to understand and\\or to follow the procedures of the study, * tea intake during the trail