Epidermodysplasia verruciformis

Epidermodesplasia verrucifomal (ectodermal dysplasia of D-cells of the skin) is a congenital skin disease that manifests itself differently in each child. As the skin matures, the symptoms worsen. For many people, symptoms may disappear completely in adulthood.

Epidermodesplasia verruciforma is also recognized as a dermatological syndrome, which includes many diseases prone to heredity and associated with the DQB1*0301 genome. However, specific genes are involved in the development, no more often than in every tenth case of this syndrome. The disease is more common in

The term "epidermodysplasia Verrutzfurm" should be established as a diagnosis. It is not permissible to use this term as a synonym for the concept of “Gero-Still-Darier syndrome”.

Epidermodysplasia veruciformis (edemamatous urticaria) is a rare hereditary skin disease of viral etiology. A type of vulgar squamous cell carcinoma. It has great diagnostic difficulties, since it is characterized by variability in manifestations and forms of progression. It occurs due to a mutation in the CCL21 gene, a receptor from the CCL-4–C9 family, which is encoded by the IL36Ra gene. A rare skin pathology characterized by rashes in the form of inflammatory elements in the form of papules, nodular thickenings or spots and the presence of atypical cells in the spinous layer of the skin. Clinically, it is similar to Downer's syndrome. According to ICD-10: B08. Epidermatodysplasia (ED)

Diagnostics. The diagnosis is made based on the history and clinical picture of skin lesions. When such spots are scraped, a mushroom-shaped pink layer is revealed, upon removal of which the microspecimen remains unchanged. Changed cells with characteristic features are subjected to histological examination. Depending on the form of the lesion, immunohistochemical analysis of the biopsy material is performed.