Pemphigus familial benign chronic: causes, symptoms, diagnosis, treatment

The bladder is a family benign chronic (synovial Guzhero-Hailey-Hailey disease) - an autosomal dominant inherited disease characterized by the appearance in the puberty period, but often later, of multiple flat blisters rapidly sticking together and opening up with the formation of erosions in the neck, armpits, perineum, inguinal folds, around the navel, under the mammary glands, often with cracks and vegetations. After healing, hyperpigmentation remains. The course of pemphigus is family benign chronic chronic, recurrent. There are atypical variants of this disease - with isolated lesions of genital organs, perianal and inguinal-femoral region, in the form of linear acantholytic dermatosis. In atypical cases, a pathomorphological study is of particular importance for diagnosis,

Pathomorphology of pemphigus is family benign chronic. In the fresh elements of the rash, a similar histological pattern is observed with Darrier's disease: they exhibit a sub-basal acantholysis with the formation of cracks or lacunae, and in more developed elements - blisters. A characteristic feature is the formation of papillary outgrowths of the dermis protruding into the cavity of the bladder. Individual acantholytic cells or their groups are found in the cavity of the bladder.

Electron microscopic examination reveals a characteristic picture of structural changes different from Darje's disease: in basal epitheliocytes forming lacunar bases, bundles of tonofilamentes are located chaotically, intercellular edema is expressed. On the surface of epithelial cells, many cytoplasmic outgrowths are revealed - microvilli, unlike ordinary microvilli they are thinned, elongated, branched. The number of desmosomes is reduced or absent, there is a lysis of their terminal sections and division into two halves. Acantholytic cells, especially immediately after their separation, retain microvilli, the tonofilament condenses around the nucleus, are deprived of contact with desmosomes. They contain well-developed organelles, signs of dystrophy are absent, which distinguishes them from acantholytic cells in normal pemphigus. In the spinous layer the condensation of the tonofilament is expressed more sharply, they are thickened, collected in large bundles, sometimes twisted in a spiral. Granular epitheliocytes contain a few immature keratogialin granules of round or oval shape that are not associated with tonofilaments. Horny flakes contain nuclei and organelles, which indicates incomplete keratinization. In dyseratotic cells, as in the case of vegetative follicular dyskeratosis, condensation of tonofilament occurs, keratogialin granules are absent.

Histogenesis of pemphigus is family benign chronic. On the basis of electron microscopic data, some authors believe that acantholysis in this disease is due to the insufficiency of cell adhesion as a result of changes in the surface properties of the cell membrane of epithelial cells, which morphologically manifests by the formation of a large number of microvilli, others - that the basis of acantholysis is the defect of the tonofilament-desmosome complex, with Darya's disease.

[1], [2], [3], [4], [5], [6], [7]