keratoderma - a collective name for a large group of dermatoses characterized by disorders of keratinization of the skin.Most often, the disease process is localized in the soles and palms.Keratoderma of palms proves thickening of the horny layer, the dead skin areas are usually clearly defined outline lilac-purple color when they themselves become horny sites brownish-yellow, hard and cracked.After some time, the tissue under the deformed portion of skin die.
addition keratoderma characterized palms noticeable changes in the shape of fingers.They sharpened considerably, nail plate thickening, the appearance on the surface noticeable bumps.On Is thickened cornified layer appears quite often bleeding deep cracks.Patients with plantar keratoderma walking quite often complain of severe pain.
Causes development of dermatological diseases have not yet fully elucidated, but experts leading role yet give specific mutations in the genes.Possible triggers the development keratoderma fact
By focal form of the disease is a linear keratoderma Fuchs limited keratoderma Bryuauera-Frantseshesti, akrokeratoelastoidoz Bones spotted disseminated keratoderma Buschke-Fischer.
To diffuse form of the disease include: Meleda keratoderma, Unna-keratoderma toast mutiliruyuschaya keratoderma, keratoderma Papillons-Lefebvre.
Also, this condition is called hereditary mutiliruyuschaya keratome syndrome Fonvinkelya.Mutiliruyuschaya keratoderma is inherited in an autosomal dominant pattern kind of diffuse keratoderma.Typically, this condition develops in the second year of life, manifested diffuse horny formations on the skin of the palms and soles, with severe hyperhidrosis.After a certain period of time to observe the formation of furrows on the fingers braid, which often leads to contractures and spontaneous amputation of the affected fingers.In the area of the knee and / or elbow joints and on the dorsum of the hand develops pronounced keratosis pilaris.There have degenerative changes in the nail plate, usually on the type of "time windows".Often, when this state there is hearing loss, alopecia and hypogonadism ruby.
This kind of disease is inherited autosomal recessive and has a second name - palmoplantar hyperkeratosis, which is often combined with periodontitis.Most often, the disease manifests itself by the third year of life.By symptomatology it is similar to the shape Meleda, but the added characteristic changes of the teeth (rapidly progressive periodontitis, gingivitis, caries, abnormal dentition).
Most often this form of the disease develops in the age interval between fifteen and twenty years.This condition is inherited in an autosomal recessive and has the following synonyms: progradiently hereditary palmoplantar keratoses anyone transgradientny palmoplantar keratosis Siemens, congenital progressive akrokeratoma, Meleda disease.This form of keratoderma seen a yellow-brown thick horny layers, the surface of which is covered with deep cracks.The entire perimeter of the lesion clearly visible violet purple outline.Often pathological process moves to the lower leg, forearm, the back surface of the feet and hands.Almost all patients have local hyperhidrosis, which leads to the formation on the surfaces of the feet and palms distinct blackheads (clogged ducts of sweat glands).Deformed nail plate and thicken.
keratoderma, Unna Toast
This form is transmitted in an autosomal dominant pattern, most often develops in the first two years of life and has the following synonyms: Toast-Unna Syndrome, congenital ichthyosis soles and palms.For this form of the disease characterized by excessive diffuse actinic skin soles and palms (there were cases when only the soles).Abnormal skin process always starts with a slight thickening of the skin of palms and soles, which resembles the bluish-brownish stripes leaf-erythema, located on the border with healthy skin.With the passage of time on the surface of the abnormal areas appear yellowish smooth horny layers.Sometimes the lesions can migrate to the back surface of the fingers or wrists.It is rarely observed in patients with the formation of deep and surface cracks, and local hyperhidrosis.In addition, when this state has been described isolated cases of destruction of teeth, nails (marked thickening) and hair, which combine with various pathologies of internal organs and skeletal abnormalities.
All keratoderma characterized by a large number of negative consequences.In the first place there is a pathological actinic skin on the soles and palms.In addition, some forms of the disease provokes development of various pathological processes in the internal organs, as well as disorders of the nervous and endocrine systems.In addition, certain types of keratoderma lead to characteristic diseases of the teeth, which often entails their premature loss.Pathological degenerative changes not only exposed skin, nails and hair.It has been described cases where keratoderma combined with oncological disease like cancer of the esophagus.
Diagnosis of the disease is based on characteristic clinical findings and histologic studies.
Treatment keratoderma is a lengthy process that often takes several years.The entire period of treatment the patient should take a certain doctor scheme B vitamins, ascorbic acid, vitamin E and A. In general
treatment of all forms of keratoderma shows the use neotigazon.Dosage depends on the severity of the pathological process and ranges from 0.3 to 1 milligram per 1 kg of body weight of the patient.In the absence neotigazon recommended for prolonged use of vitamin A in a dosage 100000-300000 mg per day.
External treatment keratoderma is to impose the use of special creams and ointments to exfoliate.In conjunction with this show the use of physical therapy.When keradermii Papillons-Lefebvre is a mandatory oral hygiene and antibiotics.
Many doctors are the basis of treatment of this disease are taking medication Retinol.However, it should appoint a special care, as in the case of incorrectly calculated dosage, or by the individual characteristics of the organism may develop side effects such as an increase in cerebrospinal fluid pressure and hypervitaminosis A.
Unfortunately, even though the use of combined treatment withthe most modern of drugs, a complete cure of the disease can not be achieved and the final recovery does not occur.However, in the event of timely and the most important thing to choose the right treatment, it is possible to markedly lessen the symptoms of the disease and to a minimum to reduce the discomfort caused by keratoderma.