Pemphigus is hereditary( congenital bullous epidermolysis)

Hereditary pemphigus is a large( more than 20 variants) group of noninflammatory diseases characterized by the tendency of the skin and mucous to develop blisters, mainly at places of mechanical trauma, even insignificant( friction, pressure, solid food intake).The disease is genetically heterogeneous, inherited both by autosomal recessive and autosomal dominant type.

The main feature of the whole group of diseases is the appearance of blisters at the slightest mechanical trauma, and not spontaneously and rarely. The disease begins with the first days of life, less often - at a later age. In the period of newborns, all forms of the disease are difficult, often lethal outcomes. The entire skin can be affected, but in most cases the rash is located on the limbs. With a simple( non-scarring) bullous epidermolysis, blisters on the skin and mucous membrane of the mouth cavity heal without scarring. Quite often, especially with the herpetiform form, excessive keratinization of the skin develops in the palms and soles. With more severe bullous epidermolysis, scars and cysts develop on the site of the healed blisters, the healing of erosions is slow. The formation of scars on the oral mucosa leads to a restriction of the mobility of the tongue, atrophy of its papillae, a decrease in the size of the oral slit, on the mucosa of the esophagus, to the development of cicatricial narrowing, and in some cases to obstruction. Re-emergence of blisters on the hands and feet leads to scarring of the fingers and subsequent destruction of the bone tissue, which is the cause of the early disability. Dystrophic changes in nails and teeth, a lag in growth and development are observed.

Prognosis for recovery is unfavorable.

Treatment of is aimed at eliminating the symptoms of the disease. Antibacterial drugs, agents that accelerate healing, vitamin E in high doses, and vitamin A in usual dosages are prescribed. For dystrophic forms of the disease, diphenine is taken twice a day at a dose of 8-10 mg / kg of body weight in children and 3.5 mg / kg in adults. Antenatal diagnosis is performed only if there are certain indications.