Erythroderma Ichthyosiform Congenital Nonbullous

Erythroderma ichthyosiformis congenita nonbullous (or also known as ichthyosis lamellar) is a rare genetic skin disorder characterized by dry, scaly, and inflamed skin throughout the body. This hereditary disease is caused by a mutation in the genes responsible for the formation of the stratum corneum of the skin.

In erythroderma ichthyosiformis congenita nonbullosa, the skin usually appears red, dry, and scaly. Large yellowish or brownish scales cover the surface of the skin, resembling flaps. This special type of skin peeling is due to a disruption in the renewal process of epidermal cells and improper formation of the stratum corneum.

Symptoms of erythroderma ichthyosiformis congenita nonbullosa may include severe itching, dry eyes and mouth, cracked skin, limited joint mobility, and increased sensitivity of the skin to injury. Some patients may have restrictions in growth and development, as well as problems with vision and hearing.

The diagnosis of erythroderma ichthyosiformis congenita nonbullous is usually made on the basis of a clinical examination, examination of the patient's medical and family history, and genetic testing. A skin biopsy may be required to confirm the diagnosis.

Treatment for this condition is aimed at relieving symptoms and maintaining the skin at optimal hydration. Patients are advised to regularly use skin moisturizers and emollients, as well as topical medications to help soften flaking and reduce inflammation. In some cases, systemic medications, including retinoids, may be prescribed to improve the skin condition.

In addition, it is important to provide patients with emotional support and education about the disease, as it can significantly impact their quality of life. Regular consultations with a dermatologist and other specialists may be necessary for effective disease management and preventive skin care.

Although congenital nonbullous erythroderma ichthyosiformis is a chronic disease, modern treatment and care methods allow patients to improve their quality of life and cope with most of the symptoms of this condition.

Description of the disease

Ichthyosiform congenital nonbullous erythroderma is a skin pathology that occurs due to an anatomical defect in the external parts of the body, manifested by significant redness and peeling of the skin.

Characteristic signs

For this skin lesion