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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
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| right controlled against moisturizing cream | Experimental |
| |
| left controlled against moisturizing cream | Experimental |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| apply VEREGEN ® 10 % on a randomized area and the moisturizing cream of the other side | Drug | After inclusion, the localization of test area will be decided and the side to treat with VEREGEN 10% will be randomized. Patient will be seen every week for 4 weeks for clinical evaluation of assessment criteria by an independent assessor. According to the randomisation he will apply VEREGEN ® 10 % on a randomized area and the moisturizing cream of the other side. If there is an improvement of at least a test zone he will enter in the follow-up period for 8 weeks. |
| Measure | Description | Time Frame |
|---|---|---|
| action and the tolerance of a daily application of topical Polyphénon E 10% ® | 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. | 4 weeks |
| Measure | Description | Time Frame |
|---|---|---|
| severity of the palmar and plantar involvement | J28 | |
| level of pruritus | until J28 | |
| global tolerance and acceptability by the patient of the Polyphénon E ® ointment |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Affiliation | Role |
|---|---|---|
| Chiaverini Christine, Dr | CHU de Nice - Service de dermatologie | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Service de dermatologie CHU de Nice | Nice | France | ||||
| Toulouse University Hospital, Dermatology Department |
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|
| apply VEREGEN ® 10 % on a randomized area and the moisturizing cream of the other side | Drug | After inclusion, the localization of test area will be decided and the side to treat with VEREGEN 10% will be randomized. Patient will be seen every week for 4 weeks for clinical evaluation of assessment criteria by an independent assessor. According to the randomisation he will apply VEREGEN ® 10 % on a randomized area and the moisturizing cream of the other side. If there is an improvement of at least a test zone he will enter in the follow-up period for 8 weeks. |
|
| J28 |
| Relapse | J84 |
| Toulouse |
| France |
| ID | Term |
|---|---|
| D017490 | Ichthyosis, Lamellar |
| ID | Term |
|---|---|
| D016113 | Ichthyosiform Erythroderma, Congenital |
| D007057 | Ichthyosis |
| D012868 | Skin Abnormalities |
| D000013 | Congenital Abnormalities |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D012873 | Skin Diseases, Genetic |
| D030342 | Genetic Diseases, Inborn |
| D007232 | Infant, Newborn, Diseases |
| D007642 | Keratosis |
| D012871 | Skin Diseases |
| D017437 | Skin and Connective Tissue Diseases |
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