Evaluate the Evolution of Body and Scalp Skin Discomfort in Patients With Hereditary Ichthyosis After Wraps (EnvelopIchtyose)

Evaluate the Evolution of Body and Scalp Skin Discomfort in Patients With Hereditary Ichthyosis After Wraps (EnvelopIchtyose)

Evaluate the Evolution of Cutaneous Discomfort of the Body and Scalp After 3 Wraps, in Patients With Moderate to Very Severe Hereditary Ichthyosis

Ichthyosis is a group of rare and chronic genetic diseases beginning at birth, in which the patient's skin is covered with scales of variable appearance and severity. This disease is disabling, in particular due to sensations of pruritus, skin pain and the possible presence of associated extra-cutaneous abnormalities. The treatment is symptomatic and is based on the daily application of moisturizing creams with a high lipid content for better effectiveness, keratolytic creams, and for severe forms, oral retinoids may be necessary. Nevertheless, the effectiveness of these treatments is limited and are considered very painful by the patients. Wraps are local treatments carried out by specialized nurses and which consist in, following a therapeutic bath, applying a large quantity of cream to the whole body, followed by an occlusion. This type of care is simple to perform, but requires nursing expertise, to date, it is not carried out in standard practice in France, which is why we wish to evaluate the effect of wraps on different parameters reflecting the skin condition in the short and medium term.

Ichthyosis is a group of rare and chronic genetic diseases beginning at birth, in which the patient's skin is covered with scales of variable appearance and severity. There are 2 distinct typical profiles: patients with thick scales without erythroderma and patients with finer scales on an erythrodermic background. This disease is disabling, in particular due to sensations of pruritus, skin pain and the possible presence of associated extra-cutaneous abnormalities. The treatment is symptomatic and is based on the daily application of moisturizing creams with a high lipid content for better effectiveness, keratolytic creams, and for severe forms, oral retinoids may be necessary. The creams improve the condition of the skin and reduce the sensations of skin discomfort, pain and pruritus, nevertheless, the effectiveness of these treatments is limited and are considered very painful by the patients. Wraps are local treatments carried out by specialized nurses and which consist in, following a therapeutic bath, applying a large quantity of cream to the whole body, followed by an occlusion. The type of cream is adapted to the area of application: thus, an emollient cream is used for the body; a cream enriched with keratolytic active ingredients is used for the feet in the event of hyperkeratosis, and an emollient cream possibly enriched with keratolytic active ingredients is used for the scalp, followed by an exposure time under a steam helmet to remove scales. This type of care is simple to perform, but requires nursing expertise, to date, it is not carried out in standard practice in France, except in the centers of Toulouse, Bordeaux and Necker and at the thermal cure of Avène-les-Bains. However, the interest of such a treatment has never been evaluated independently, which is why in this study we want to evaluate the effect of carrying out a daily treatment (including a therapeutic bath, a wrap (body and feet) and scalp care) over 3 consecutive days, on different parameters reflecting the skin condition in the short and medium term

Interventional, prospective, multicenter (Toulouse - Bordeaux - Paris Necker), comparative (baseline vs. short and medium term)

The skin care consisting of wraps (body and feet) and scalp care, will be carried out consecutively over 3 days, in the hospital (outpatient or in day/traditional hospitalization).

The topical treatment that will be used in this study are: For the body, an emollient cream with a high lipid content: Cold cream For the scalp and feet, an emollient preparation with keratolytic action: 10% or 30% urea, depending on the scalp and feet damage Each of the 3 treatments (body, scalp and feet) will be carried out as follows: Application of different topicals all over the body and face, after taking a lukewarm therapeutic bath of 20 minutes Occlusion by "wrapping" the patient in a plastic film, then covering with a blanket After 1 hour, the unabsorbed excess cream is removed with a towel

Percentage of patients showing a reduction of at least 3 points of their skin discomfort in the entire body, measured with the AVS scale (0-10) of cutaneous discomfort between baseline and 11 days after treatment

Percentage of patients showing a reduction of at least 3 points of their skin discomfort in the entire body, measured with the Analogue Visual Scale (AVS) (0-10) of cutaneous discomfort between baseline and day 4

Percentage of patients showing a reduction of at least 3 points of their skin discomfort in the entire body, measured with the Analogue Visual Scale (AVS) (0-10) of cutaneous discomfort between baseline and day 30

Variation of erythema and scales severity judged by : - Their intensity, measured with the Visual Index for Ichthyosis Severity (VIIS)

Variation of erythema and scales severity judged by : -Their extent, measured with the scores A and B of the Specific Clinical Ichthyosis Score

Percentage of patients showing a reduction of at least 3 points of their skin discomfort in the feet and scalp, measured with the Analogue Visual Scale (0-10) of cutaneous discomfort

Percentage of patients showing a reduction of at least 3 points of their skin discomfort in the feet and scalp, measured with the Analogue Visual Scale (0-10) of cutaneous discomfort

Percentage of patients showing a reduction of at least 3 points of their skin discomfort in the feet and scalp, measured with the Analogue Visual Scale (0-10) of cutaneous discomfort

Variation of patient's quality of life, measured by the ichthyosis specific quality of life questionnaire (IQoL-32)

Inclusion Criteria: Patient with hereditary ichthyosis regardless of the clinical form Patient with a scaling or erythema score > 6/16 Patient with a skin discomfort score > 3/10 Patient having given written, free and informed consent Patient affiliated to a social security scheme Exclusion Criteria: Patient with a skin disease other than ichthyosis Patient having modified his treatments for ichthyosis in the month preceding inclusion Patient with known intolerance or allergy to one of the topicals used in the study Patient unable to complete the study questionnaires Patient under a protection regime