Keratodermia

Description:

Keratodermias - group of the dermatosis which is characterized by disturbance of processes of keratinization - excess cornification preferential palms and soles.

Reasons keratodermia:

The reasons and pathogeny of a disease are finally not found out. By researches it is established that keratodermias are caused by mutations in the genes coding a keratin 6, 9, 16. In a pathogeny hormonal dysfunctions, first of all gonads, bacterial and viral infections are of great importance insufficiency of vitamin A. They are one of symptoms of hereditary diseases and tumors of internals (perapsoriatichesky keratodermias).

Keratodermia symptoms:

Distinguish diffusion (a keratodermia of Unny-Tosta, Meled's keratodermia, Papiyona-Lefebvre's keratodermia, a mutiliruyushy keratodermia and syndromes including a diffusion keratodermia as one of the main symptoms) and focal (the disseminated spotty keratodermia of Fischer-Bushke, акрокератоэластоидоз Kostya, a limited keratodermia of Bryuaauera-Frantseshesti, a linear keratodermia of Fuchs, etc.) keratodermias.

Keratodermia of Uiny-Tosta (synonyms: the inborn ichthyosis of palms and soles, a syndrome of Uiny-Tosta) is transferred autosomal is prepotent. Diffusion excess keratinization of skin of palms and soles (sometimes only soles) which develops in the first two years of life is noted. Skin and pathological process begins with an easy thickening of skin of palms and soles in a look a strip of an erythema of lividny color on border with healthy skin. Over time pases of their surface appear smooth, yellowish color horn stratifications. Defeat seldom passes a pas a dorsum of wrists or fingers. At some patients superficial or deep cracks can be formed and the local hyperhidrosis is noted. At the patient observed by the author the uncle on the mother's side, the brother and the son suffered from a keratodermia of Uiny-Tosta.

Cases of damage of nails (thickening), teeth, hair are described at a keratodermia of Uiny-Tosta in combination with various anomalies of a skeleton and щ pathology of internals, nervous and endocrine systems.

Histopathology. At a histologic research find the expressed hyperkeratosis, гранулез, an acanthosis, small inflammatory infiltrates in an upper layer of a derma. Differential diagnosis. The disease should be differentiated from a keratodermia of other types.

Meled's keratodermia (synonyms: Meled's disease, the inborn progressing akrokeratoma, a palmar and bottom transgradient keratosis of Siemens, a hereditary palmar and bottom pro-gradient keratosis of Kogoya) is descended autosomal рецессивио. At this form of a keratodermia thick horn stratifications of yellowy-brown color with deep cracks appear. At the edges of the center of defeat the violet-lilac border width in several millimeters is visible. Transition of process to a dorsum of brushes and feet, forearms and shins is characteristic. At most of patients the local hyperhidrosis is observed. In this regard the surface of palms and soles becomes slightly wet and becomes covered by black points (output channels of sweat glands).

The disease can develop by 15-20 years. Nails are thickened, deformed.

Histopathology. At a histologic research reveal a hyperkeratosis, sometimes - an acanthosis, in a papillary layer of a derma - chronic inflammatory infiltrate.

Differential diagnosis. Shallowed a keratodermia it is necessary to distinguish from a keratodermia of Unny-Tosta.

Papiyona-Lefebvre's keratodermia (synonym: the palmar and bottom hyperkeratosis with periodontitis) is inherited autosomal рецессивно.

The disease is shown on 2-3 year of life. The clinical picture of a disease is similar to a disease Shallowed. Besides, changes of teeth are characteristic (anomalies of eruption of milk and second teeth with development of caries, an ulitis, quickly progressing paradontosis with premature dedentition).

Histopathology. At a histologic research reveal a thickening of all layers of epidermis, especially horn, in a derma - insignificant cellular accumulations of lymphocytes and histiocytes.

Differential diagnosis. The disease should be distinguished from other keratodermias. At the same time the important distinctive point is the characteristic pathology of teeth which is not found at other forms of hereditary diffusion keratodermias.

Mutiliruyushchy keratodermia (synonyms: Fonvinkel's syndrome, a hereditary mutiliruyushchy keratoma) - a version diffusion keratodermias, inherited autosomal it is prepotent. Develops on the 2nd year of life, it is characterized by diffusion horn stratifications on skin of palms and soles with a hyperhidrosis. Over time shnurovidny furrows on fingers form that leads to contractures and spontaneous amputation of fingers. On a dorsum of brushes, and also in the field of elbow and knee joints the White's disease is expressed. Nail plates are changed (often as hour glasses). Cases of a hypogonadism, ruby baldness, a hearing loss, a pachyonychia are described.

Histopathology. At a histologic research reveal a powerful hyperkeratosis, гранулез, an acanthosis, in a derma - the small inflammatory infiltrates consisting of lymphocytes and histiocytes.

Differential diagnosis. At differentiation of a mutiliruyushchy keratodermia from other forms diffusion the keratodermia should consider first of all effect of mutilation, uncharacteristic for other forms. Carrying out differential diagnosis of all forms of a diffusion keratodermia, it is necessary to remember that it can be one of the main symptoms of a number of hereditary syndromes.

Treatment of a keratodermia:

In the general therapy of a keratodermia it is shown неотигазон. The dose of drug depends on weight of process and makes 0,3-1 mg/kg of weight of the patient. In the absence of a neotigazon recommend vitamin A in a dose from 100 to 300 000 mg a day a long time. Outside therapy consists in use of ointments with aromatic retinoids, keratolytic and steroid means.