Ichthyosis Inherited

This study looks at whether a cream containing 10% cholesterol works better than a cream containing 10% urea for people with autosomal recessive congenital ichthyosis (ARCI), a rare inherited skin condition that causes dry, thickened, scaly skin from birth. People with ARCI have a weakened skin barrier because their skin cannot properly produce or arrange the natural fats (lipids) needed to keep moisture in and irritants out. This leads to excessive water loss through the skin, dryness, itching, cracking, and a higher risk of skin infections. The current standard treatment is urea cream, which helps soften and remove scale but does not repair the underlying fat structure of the skin barrier. Cholesterol is one of the key natural fats that makes up a healthy skin barrier, so applying it directly to the skin may help rebuild that barrier more effectively than urea. In this study, each participant will have one cream applied to one side of the body and the other cream applied to the matching area on the opposite side, so that each person acts as their own comparison. Researchers will measure how much water the skin loses (transepidermal water loss), skin hydration, and skin pH before and after treatment. They will also assess dryness and itching, take standardized clinical photographs, and ask participants to complete quality-of-life questionnaires (the Dermatology Life Quality Index, or its children's version, the Children's Dermatology Life Quality Index) as well as a satisfaction questionnaire for each cream. The goal is to find out whether cholesterol cream restores the skin barrier better than urea cream, reduces dryness and itching more effectively, and leads to a better quality of life for people living with ARCI. The results may help guide more targeted skin care recommendations for this rare genetic condition. This study is being conducted at the Institute of Dermatology, Bangkok, Thailand, and has been approved by the Institute's Ethics Committee.

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.