Polymorphic photodermatosis clinically combines the features of solar prurigo and eczema arising from exposure to the sun. Disease, in general, develops under the influence of UVB, sometimes UVA rays.
The existence of porphyrin substance and the violation of its metabolism were discovered more than 100 years ago. N. Guntcr (1901) called diseases that occur with a disruption of porphyrin metabolism, "hemoporphyria", and J. Waldenstrom (1937) by the term "porphyria".
For the first time Cazenave (1856) described the Morrow-Brook follicular keratosis called "acnae sébacée cornu". Then N. A. Brook and P. A Morrow, after studying the clinical course of the disease, proposed the term "follicular keratosis".
Pachyonikhia congenital is a variant of ecto-mesodermal dysplasia. Inheritance is heterogeneous, autosomal recessive, linked to the sex. Men are more often ill. The causes and pathogenesis of the congenital pachyonhia are unclear. In urine there is a high level of hydroxyproline.
Angiomatosis is a hereditary family hemorrhagic - an autosomal dominant disease. There are several reports in the literature that the disease has been detected in several generations.
The causes and pathogenesis of juvenile polyfibromatosis of the Rhine's fingers have not been fully established. It is believed that dermatosis has an autosomal dominant type of inheritance.
Family acroheria (Gottron's syndrome) is a rare disease described in 1941 by N. Gottron. The causes and pathogenesis of the acroheria of the family (Gottron's syndrome) are not fully understood. In the development of the disease, an important role is played by the disruption of the structure and function of fibroblasts and collagen synthesis, hypofunction of the pituitary gland. There are reports of family cases of the disease.
Tuberous sclerosis is a hereditary disease characterized by hyperplasia of ecto- and mesodermal derivatives. Type of inheritance is autosomal dominant. Mutant genes are located in the loci 16p 13 and 9q34 and encode tuberins - proteins that regulate the GT-phase activity of other extracellular proteins.
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 pigment incontinence (Bloch-Sulzberg melanoblastosis). Incontinence of the pigment is due to a mutant dominant gene localized in the X chromosome.
