Poikiloderma Hereditary Sclerosing

Hereditary sclerosing poikilodermatia (synonyms - sclerosing poikilodysplastic epidermolysis, syndactylic poikilosclerosis, poikilosclerotic poikiloderma, 3rd form of hyperkeratotic ichthyosis, Thomson syndrome, 2nd Foster Cohn type, elephantine foot feet, elephantiasis obliterans, Klipper and Frakel disease, palmoplantar keratodermatosis with syndrome Rapa, sesia) is a recessive genetic disease of the skin, autosomal recessive, which is characterized by persistent disruption of the regenerative processes of epithelial cells in the layers of the skin and mucous membranes. More common in men.

The basis of diagnostic

Poikiloderma hereditary Sclerosis is a rare genetic skin disease characterized by the appearance of crusts and peeling of the skin, and scarring. This diagnosis refers to hereditary diseases, but it can also be caused by various factors such as injury, infection and other skin diseases.

Poikiloderma hereditary sclerosing disease is hereditary and can manifest itself at different ages, from the prenatal period until the child reaches adulthood.

Symptoms of hereditary poikiloderma are observed in every tenth patient with hereditary metabolic diseases. Thomson syndrome is a rare inherited skin disease. The first signs your child may show are dry skin, which may peel or crack. This disease is associated with the inferiority of the synthesis of certain compounds in the skin. A skin defect appears in the form of changes in the color of the skin from white to gray and bluish

Poikiloderma hereditary sclerosis is a rare inherited disease that is characterized by the appearance of unusual flat and raised elements of various sizes on the skin. These elements can cause an itching or burning sensation, which can lead to pain and difficulty moving. In this condition, the skin appears uneven and wrinkled, and some elements may be more stubborn than others. Often these elements remain on the skin permanently, although they may disappear in certain areas of the skin. In addition, there are cases where poikiloderma becomes more pronounced over time. Although poikiloderma is rare, it can cause significant physical discomfort and psychological impact.

Hereditary sclerosing poikiloderthia is also known as Thomson's syndrome. This condition is usually caused by certain genetic mutations that cause the formation of certain proteins in the skin to fail, resulting in these characteristic skin features. Sclerotic poikiloderic manifestations can develop at any age, starting in childhood and then continuing to increase in size throughout life.

Treatment for this condition depends on the severity of symptoms and may include the use of local anesthetics to reduce pain and itching, as well as systemic therapy to reduce inflammation and fluid retention. Treatment may also be carried out with medications aimed at reducing the formation of excess skin and increasing the elasticity of the dermis.

One of the biggest challenges for patients with poikiloderma hereditary sclerosing is the severe loss of quality of life as a result of physical pain, the constant presence of skin manifestations, and the associated psychological consequences. People are often forced to hide from others due to lack of self-confidence, bad mood and stress related to the appearance of their skin. Some patients may experience social and occupational difficulties due to their deficits and treatment-related dependent behavior.

In general, poikiloderma hereditary sclerosis is a serious and very rare disease, the causes of which remain unknown today. However, many cases of poikilodermic manifestations remain untreated, which further complicates the lives of patients and requires increased attention from medical personnel.