(epidermolysis bullosa; Greek epi-on, atop + derma skin + lysis destruction, dissolution; lat. bulla water bubble; synonym: inborn violent Lise's epidermis, an inborn pemphigus, a violent acantolysis, inborn violent dystrophy, a traumatic violent dermatosis) — the rare hereditary chronic disease of skin which is characterized by emergence of bubbles in the places, a thicket which are exposed to pressure and friction.
Distinguish two main forms E. — dystrophic, described by Fox (W. T. Fox) in 1879, and the simple, described A. J. Goldscheider in 1882. Simple form E. it is noted more often and it is inherited on autosomal to - minantnomu to type; dystrophic form E. 6. can have autosomal and dominant or autosomal retses-sivny a mode of inheritance.
At a dystrophic form of a disease bubbles are located preferential between epidermis y with a derma (a subepidermis lno), at a simple form — vnutriepidermal-but in a malpigiyevy layer of epidermis (acanthceous and basal layers of epidermis), is more rare in horn. They result from disintegration of cells of a basal layer of epidermis, in to-rykh the large vacuoles merging then in intra epidermal bubbles form. Under a bubble nipples of a derma are maleficiated. At both forms in an upper part of a derma circulatory and absorbent vessels are expanded, puffiness of fabric and diffusion, preferential lymphocytic, infiltrate of different intensity is noted. At a dystrophic form elastic fibers in a derma are usually rarefied or completely are absent, and in the atrophic sites remaining on site rashes, they are not recovered further. In some cases changes of elastic fibers are defined in externally not changed skin. At a research of the urtikaropodob-ny hems which sometimes are formed on skin at a dystrophic form without the previous clinically noticeable bubbles, a subepidermis microbubbles are lno found.
Both the simple, and dystrophic form of a disease begin usually after the birth and reach the expressed development when the child begins to creep and go. Bubbles of different size appear on externally not changed skin, hl. obr. on extensor surfaces of extremities, especially in joints, and also on brushes, feet, a neck, a waist. They are filled with transparent or hemorrhagic contents, their emergence is followed by the insignificant inflammatory phenomena. In several days bubbles are opened, forming erosion, or shrink in crusts.
At a simple form E. erosion on places of bubbles are quickly epithelized. Usually by the period of puberty process calms down. Abortal kind of a simple form is the epidermolysis violent Weber — Kokkeyna who is characterized by defeat only of feet (sometimes brushes), recuring in warm season and followed by a local hyperhidrosis.
At a dystrophic form, edges proceeds more hard, after falling away of crusts on site of the shrunk bubbles there are atrophic hems, against the background of to-rykh the epidermal cysts reminding mi-lium (see) develop; dystrophy of nails is observed (see), the union of fingers, a resorption of a bone tissue of separate phalanxes, deformation of brushes and feet, sometimes in combination with dystrophy of teeth and hair are possible (polidisplastiche-Skye a kind of a dystrophic form E.). Symptom of epidermal amotio (amotio of epidermis in the fringe region of an erosion at a pandiculation tweezers for a tire of a bubble) often positive. Approximately at a half of patients bubbles arise on a mucous membrane of an oral cavity, especially in language. On these places rough vicious hands form, on to-rykh there can be bubbles again. Most actively process proceeds at early children's age and at the age of
12 — 15 years.
Version dystrophic E. are albopapuloidny E., shown peculiar white urtikaropodobny hems on site of the former bubbles, but sometimes arising and it is spontaneous. Also localized forms E are described. Rare version dystrophic E. is ulcer and vegetans E., at Krom on site of bubbles resistant ulcerations with vegetations appear. Allocate also lethal form E., which meets seldom; it arises during the first hours after the birth and is characterized by peeling of big sites of epidermis in the places which are exposed to traumatizing; children perish in the first weeks or months of life.
The diagnosis establish on the basis a wedge, the picture confirmed in doubtful cases with data gistol. researches.
Differential diagnosis is carried out with various forms of a violent dermatosis (see the table to St. Pemphigus, t. 21, p. 382).
Treatment includes purpose of vitamins A, With, P, groups B, gamma-globulin, phytin, drugs of calcium, iron, plasma transfusion, at hard proceeding dystrophic forms E. use of corticosteroids is shown. Topical treatment consists in opening of bubbles and drawing on the erosive surfaces of the disinfecting and epithelizing ointments.
The forecast concerning life, as a rule, favorable. At a dystrophic form in hard cases there can come disability in connection with rough deformation of brushes and feet, a resorption of a bone tissue of phalanxes, an union of fingers, etc.
Prevention of an aggravation of process consists in protection of skin of the patient from injuries, and also from accession of consecutive infection. Bibliography: Differential diagnosis of skin diseases, under the editorship of. A. A. Stud-nitsina, M., 1983; Lelis I. I. and Leliyene V. A. Hereditary violent epidermolysis and its options, Vestn. dermas, and veins., No. 6, page 63, 1977; Mashkilleyson L. H. Private dermatology, page 268, M., 1965; Males V. I. Albopapuloidny violent epidermolysis, Vestn. dermas, and veins., No. 6, page 64, 1971; Studnitsin A. A., etc. Clinic and a current of a violent
epidermolysis at children, in the same place, No. 4, page 3, 1974; Dermatology in general medicine, ed., by T. B. Fitzpatrick a. o., p. 334, N. Y., 1979. A. JI. Mashkilleyson.