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Erythrokeratoderma: causes, symptoms, diagnosis, treatment

 
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Last reviewed: 23.04.2024
 
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At present, this group of erythrokeratoderma includes disorders of keratinization of the skin according to the type of hyperkeratosis and proceeding on the erythematous background. However, few dermatologists refer it to the ichthyosis group.

Causes and pathogenesis of erythrokeratoderma. The causes of the development of erythrokeratodermy have not been fully studied. According to some scientists, in the pathogenesis of erythrokeratoderma, an increasing role is played by an increase in the content of acid phosphatase in epidermal cells, the accumulation of nucleotide triphosphatase and alkaline phosphatase in the papillary layer of the dermis, and other factors. The disease is hereditary.

Symptoms. Dermatologists distinguish the following clinical forms of erythrokeratoderma: variable figurative; symmetrical progressing (Gottron's syndrome); limited symmetrical, progressing with peripheral neuropathy and deafness (Schneider's syndrome); rare, atypical, etc.

Eritrokeratodermia symmetric progressing Gottron often begins in the first years of life. At the beginning (1-3 years) the process is actively progressing, and then it is suspended and, possibly, the process regresses. The onset of the disease is also described in older and older ages.

Symptoms of erythrokeratoderma. Eritrokeratodermia symmetric progressive Gottron is characterized by the presence of hyperkeratotic rosato-red plaques with a narrow erythematous corolla or hyperpigmented margin. The rashes are located symmetrically on the skin of the knee and elbow joints, the back surface of the hands and feet. Sometimes the rash may be located on other areas of the skin. The skin of the trunk, palms and soles usually remains unaffected. Change of nails is not noted.

Histopathology. Histological examination shows hyperkeratosis, parakeratosis in places, hypergranulosis, moderate acanthosis, in the dermis - vasodilation, lymphocytic infiltrate.

Differential diagnosis. The disease should be distinguished from psoriasis, lamellar ichthyosis, etc.

Treatment. Prescribe vitamins, antioxidants, retinoids or corticosteroids inside. External - hormonal and keratolytic means. A good effect is noted from PUVA therapy.

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