Keratoderma: causes, symptoms, diagnosis, treatment

Keratoderma - a group of dermatoses characterized by a violation of the processes of keratinization, - excessive horn formation mainly of the palms and soles.

The causes and pathogenesis of the disease have not been fully elucidated. Studies have established that keratodermia are caused by mutations in the genes of keratin coding 6, 9, 16. In pathogenesis, vitamin A deficiency hormonal dysfunctions, especially the sex glands, bacterial and viral infections are of great importance. They are one of the symptoms of hereditary diseases and tumors of internal organs (parapsoric keratoderma).

Symptoms. There are diffuse (keratoderma Unna-Toast, keratoderma Meleda, keratodermia Papillon-Lefevre, mutilated keratoderma and syndromes including diffuse keratoderma as one of the main symptoms) and focal (disseminated spotted keratoderma Fisher-Buschke, acrokeratoelastoidosa Bone, limited keratodermia Bruaaouer-Franzesci, linear keratodermia Fuchs, etc.) keratoderma.

Keratoderma of Wyna-Toast (synonyms: congenital ichthyosis of the palms and soles, syndrome of Wyna-Toast) is transmitted autosomal dominantly. Diffuse excess keratinization of the skin of the palms and soles (sometimes only the soles), which develops in the first two years of life, is noted. Skin and pathological process begins with a slight thickening of the skin of the palms and soles in the form of a band of erythema livid color on the border with healthy skin. Over time, smooth, yellowish horny layers appear on their surface. The lesion rarely passes to the back surface of the wrists or fingers. Some patients may develop superficial or deep cracks and local hyperhidrosis is noted. In the case of the uncle observed by the author on the part of the mother, the brother and son suffered keratoderma of Wyna-Toast.

Cases of nail damage (thickening), teeth, hair with keratoderma of Wyna-Toast in combination with various anomalies of the skeleton and pathology of internal organs, nervous and endocrine systems are described.

Histopathology. Histological examination reveals pronounced hyperkeratosis, granulosis, acanthosis, small inflammatory infiltrates in the upper layer of the dermis. Differential diagnosis. The disease must be differentiated from keratoderma of other types.

Mélade's keratoderma (synonyms: Meled's disease, congenital progressive acrokeratoma, Simon's pediatric trans-gradient keratosis, hereditary palmar-plantar grading kogatosis of Kogoy) is inherited by autosomal recessivio. With this form of keratoderma, thick horny layers of yellow-brown color with deep cracks appear. At the edges of the lesion, a purple-purple rim several millimeters in width is visible. Characteristic is the transition of the process to the rear surface of the hands and feet, forearms and legs. Most patients have local hyperhidrosis. In this regard, the surface of the palms and soles becomes slightly moist and covered with black dots (excretory ducts sweat glands).

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

Histopathology. Histological examination reveals hyperkeratosis, sometimes - acanthosis, in the papillary layer of the dermis - chronic inflammatory infiltrate.

Differential diagnosis. Keratoderma Melle must be distinguished from keratoderma Unny-Toast.

Keratodermia Papillon-Lefevre (synonym: palmar-plantar hyperkeratosis with periodontitis) is inherited autosomally-recessively.

The disease manifests itself in 2-3 years of life. The clinical picture of the disease is similar to that of Melle. In addition, the changes in the teeth (anomalies of eruption of dairy and permanent teeth with the development of caries, gingivitis, rapidly progressing paradontosis with premature tooth loss) are characteristic.

Histopathology. Histological examination reveals a thickening of all layers of the epidermis, especially of the horny, in the dermis - insignificant cell clusters of lymphocytes and histiocytes.

Differential diagnosis. The disease should be distinguished from other keratodermias. In this case, an important distinctive feature is the characteristic pathology of the teeth, which does not occur in other forms of hereditary diffuse keratoderma.

Mutilating keratoderma (synonyms: Fonvinkel syndrome, hereditary mutating keratoma) - a kind of diffuse keratodermia, heritable autosomal dominant. It develops on the 2nd year of life, it is characterized by diffuse horny layers on the skin of the palms and soles with hyperhidrosis. Over time, cord-like grooves are formed on the fingers, which leads to contractures and spontaneous amputation of the fingers. The follicular keratosis is expressed on the back surface of the hands, as well as in the area of the elbow and knee joints. Nail plates changed (often by the type of watch glass). Cases of hypogonadism, ruby alopecia, loss of hearing, pachyonihia are described.

Histopathology. Histological examination reveals powerful hyperkeratosis, granulosis, acanthosis, in the dermis - small inflammatory infiltrates, consisting of lymphocytes and histiocytes.

Differential diagnosis. When differentiating mutating keratoderma from other forms of diffuse keratodermia, the mutation effect, uncharacteristic for other forms, should be taken into account first of all. Carrying out differential diagnostics of all forms of diffuse keratoderma, it must be remembered that it can be one of the main symptoms of a number of hereditary syndromes.

Treatment. In the general therapy of keratoderma, neotigazone is shown. The dose of the drug depends on the severity of the process and is 0.3-1 mg / kg of the patient's weight. In the absence of neotigazone recommend vitamin A at a dose of 100 to 300 000 mg per day for a long time. External therapy consists in the use of ointments with aromatic retinoids, keratolytic and steroid agents.

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