Syndromic recessive X-linked ichthyosis is a rare genetic disorder that affects the skin. It is caused by a mutation in the STS gene, which is located on the X chromosome. People with this disorder have dry, scaly skin that is often thickened and cracked. The skin may also be darker than normal in some areas. Symptoms usually appear at birth or in early childhood and can range from mild to severe. Treatment typically involves the use of topical creams and ointments to help keep the skin moisturized and reduce the symptoms.

The symptoms of Syndromic recessive X-linked ichthyosis include:

-Thick, scaly skin
-Dry, cracked skin
-Redness and inflammation
-Itching and Burning sensations
-Thickening of the skin on the palms and soles
-Thickening of the skin on the scalp, face, and neck
-Thickening of the skin around the eyes
-Thickening of the skin on the elbows and knees
-Thickening of the skin on the back and chest
-Thickening of the skin on the buttocks and thighs
-Thickening of the skin on the abdomen
-Thickening of the skin on the genitals
-Thickening of the skin on the hands and feet
-Thickening of the skin on the arms and legs

Syndromic recessive X-linked ichthyosis is caused by mutations in the gene STS (steroid sulfatase). This gene is responsible for producing an enzyme that helps to break down cholesterol sulfate, which is necessary for normal skin development. Mutations in this gene can lead to a buildup of cholesterol sulfate in the skin, resulting in the dry, scaly skin characteristic of ichthyosis.

The primary treatment for Syndromic recessive X-linked ichthyosis is topical retinoid therapy. This involves applying a topical retinoid cream or ointment to the affected areas of skin. Other treatments may include topical emollients, topical steroids, and systemic retinoids. In some cases, laser therapy may be used to reduce the appearance of scaling.

The risk factors for Syndromic recessive X-linked ichthyosis include:

1. Being male
2. Having a family history of the condition
3. Having a mutation in the STS gene
4. Having a mutation in the TGM1 gene
5. Having a mutation in the ALOX12B gene
6. Having a mutation in the ALOXE3 gene
7. Having a mutation in the ABCA12 gene
8. Having a mutation in the CYP4F22 gene

At this time, there is no cure for Syndromic recessive X-linked ichthyosis. However, there are medications and treatments available to help manage the symptoms. These include topical retinoids, emollients, and systemic retinoids. Additionally, phototherapy and laser treatments may be used to reduce scaling and improve the appearance of the skin.