Keratoderma Mutilating

Keratoderzia Mutating (Km)

**Keratoderzia Mutilating** - idiopathic inflammatory keratitoparoneid dermatitis, dystrophic necrosis of epidermocytes as a result of damage to hair follicles, paranoid vesicular destruction of the keratin layer in combination with thinning of the stratum corneum and its thickening.

There are two known variants of the course of the disease - localized, in the form of a single nodule centered in the zone of transition of the skin of the cheeks to the nose (in women more often than in men), and generalized. In the first case, the lesion usually begins in childhood. The lesion is limited to one follicle located on the skin or mucous membrane. If the lesion is localized on the inner surface of the lip, it is possible to painlessly remove it with tweezers. Subsequently, after one lesion, new ones may appear. During puberty, single groups of follicles appear, often multiple nodes are located in multiple follicles. A continuous pityriasis process is observed extremely rarely. Photosensitivity is characteristic, manifested in the form of vascular disorders on the skin and conjunctiva; ulcerations on the conjunctiva of the eyelids and cheeks are quite common.

Increased sweating, itching, diffuse swelling and discoloration of the skin of the face, head, neck and upper extremities are noted. Hyperkeratotic, dense, yellow, round nodules with a depression in the center form on the skin, slowly increasing in size. At the site of the nodule, atrophic spots are observed, the surface

Keratoderma mutilans (KM) is a rare genetic disease that manifests as changes in the skin and keratinization as a rough, scaly or keratinizing horny cutaneous pannus that partially covers the body, face, palms and soles. This disease can cause significant impairment in function and quality of life.

Keratoderma mutilata is characterized by hereditary skin lesions associated with disruption of the synthesis and normal functioning of the keratin protein. This protein is a key component of the skin and is responsible for its strength, elasticity and smoothness. In CM, normal keratin synthesis is disrupted, resulting in the formation of rough, scaly or keratinized skin that covers the body, face and extremities.

Clinical manifestations of keratoderma may include hyperkeratosis, decreased nail density, callus formation, and thickened skin. The most common symptoms are irritation and itching, which can be worsened by exposure to irritants such as water, soap and food. Nail damage and chronic keratolytic stomatitis may occur. Treatment includes the use of topical steroid creams and ointments to relieve itching and reduce irritation.

The causes of keratodermal mutation are not precisely known, but researchers associate