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. The treatment is symptomatic and is based on the daily application of moisturizing creams with a high lipid content. 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 the investigateur 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 the investigators want to evaluate the effect of carrying out a daily treatment over 3 consecutive days, on different parameters reflecting the skin condition in the short and medium term
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
34
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
Toulouse University hospital
Toulouse, France
RECRUITINGSkin discomfort Analogue Visual Scale (AVS)
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
Time frame: day 11
Reduction of skin discomfort - 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 4
Time frame: day 4
Reduction of skin discomfort - day 30
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
Time frame: day 30
Variation of erythema and scales severity - Visual Index for Ichthyosis Severity
Variation of erythema and scales severity judged by : \- Their intensity, measured with the Visual Index for Ichthyosis Severity (VIIS) : scores scored separately out of 16. The 16 point scorring is : 4 zones: upper back, upper arm, lower legs, top of foot, scored out of 4 with 0 = no lesions, 1 = areas of normal skin with small scales, 2 = confluent smoothing, 3 = confluent scales and 4 = confluent scale
Time frame: day 4
Variation of erythema and scales severity
Variation of erythema and scales severity judged by : -Their extent, measured with the scores A and B of the Specific Clinical Ichthyosis Score
Time frame: day 4
Cutaneous pain - day 4
Variation of pain assessed by the Analogue Visual Scale (0-10) of skin pain
Time frame: day 4
pruritus - day 4
Variation of pruritus assessed by the Analogue Visual Scale (0-10)
Time frame: day 4
sweating - day 4
Variation of sweating assessed by the Analogue Visual Scale (0-10)
Time frame: day 4
Cutaneous pain - day 11
Variation of pain assessed by the Analogue Visual scale (0-10) of skin pain
Time frame: day 11
pruritus - day 11
Variation of pruritus assessed by the Analogue Visual scale (0-10)
Time frame: day 11
sweating - day 11
Variation of sweating assessed by the Analogue Visual Scale scale (0-10)
Time frame: day 11
Cutaneous pain - day 30
Variation of pain assessed by the Analogue Visual Scale (0-10) of skin pain
Time frame: day 30
pruritus - day 30
Variation of pruritus assessed by the Analogue Visual Scale scale (0-10)
Time frame: day 30
sweating - day 30
Variation of sweating assessed by the Analogue Visual Scale (0-10)
Time frame: day 30
Feet and scalp discomfort - day 4
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
Time frame: day 4
Feet and scalp discomfort - day 11
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
Time frame: day 11
Feet and scalp discomfort - day 30
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
Time frame: day 30
32-item Ichthyosis Quality of Life questionnaire (IQoL-32)
Variation of patient's quality of life, measured by the ichthyosis specific quality of life questionnaire (IQoL-32)
Time frame: 30 days after treatment
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