Bubbly dermatitis

Description:

Bubbly dermatitis (pemphigus) - the disease which is shown education on uninflammed skin and mucous membranes of the bubbles developing as a result of an acantolysis and extending without adequate treatment to all integument that can lead to the death of the patient. Are ill a pemphigus of the person of both sexes, 35 years are preferential more senior. Also children can seldom be ill.
According to various authors, incidence of a pemphigus on average does not exceed 1,5% of all dermatosis.
Distinguish four clinical forms of a pemphigus: ordinary, vegetans, leaflike and seborrheal (erythematic). The mucous membrane of a mouth is surprised at all forms of a pemphigus, except leaflike. Considerably other forms the ordinary pemphigus meets more often. According to W. Lever, the ordinary pemphigus at 62% of patients begins with rashes on a mucous membrane of a mouth, and according to A. L. Mashkilleyson - nearly 85%. If the disease begins with damage of skin, then in the subsequent rashes almost always develop also on a mucous membrane of a mouth.

Symptoms Bubbly dermatitis:

The dermatosis, as a rule, begins with damage of mucous membranes of an oral cavity and a pharynx, and then skin of a trunk, extremities, inguinal and axillary hollows, persons, external genitals is involved in process. At a number of patients primary centers on skin, mucous membranes can spontaneously be epithelized and disappear, but usually soon they are formed again, and often process begins to gain widespread character quickly enough. Damage of a mucous membrane of a mouth and lips is characterized by formation of the bubbles having the thinnest tire formed by an upper part of an acanthceous layer of an epithelium. In the conditions of continuous maceration and with a pressure bubbles are instantly opened with food lumps, and on their place round or oval erosion so practically bubbles on a mucous membrane of an oral cavity at a pemphigus erosion do not manage to be seen are formed. Bright red color, are located against the background of not changed mucous membrane. On the periphery of an erosion it is possible to see bubble tire scraps at a pandiculation for which easily Nikolsky's symptom is caused. The remains of a tire of the opened bubble can cover an erosion, in this case an impression is made that the erosion is covered with a grayish-white plaque. This "plaque" easily is removed at a touch to it the pallet. If the diagnosis is timely not made and treatment is not begun, then after single erosion which are more often localized on a mucous membrane of cheeks (especially in retropainting area), the sky, the lower surface of language and in the field of a mouth floor appear new which, merging among themselves, form extensive erosive surfaces without tendency to healing. Meal and the speech become almost impossible because of pains. The specific fetid smell from a mouth is usually noted. On skin on site of bubbles in the course of evolution erosive surfaces are quickly formed becoming wet, polycyclic outlines. At regeneration of epidermis there are layered brownish crusts, and then on sites of the centers there is a brownish pigmentation. In other cases (at bent of process to spontaneous remission or under the influence of steroid therapy) tires of bubbles do not collapse as exudate resolves and tires, being gradually fallen down, turn into a thin crust which then disappears. Spontaneous remissions, as a rule, are replaced by a recurrence. At the high-quality course of process the general condition of the patient almost does not change. At a malignant current there comes the general exhaustion of an organism, are noted the septic fever which is especially expressed at accession of consecutive infection, an eosinophilia, a delay in fabrics of sodium, chlorides, protein content reduction. Changes of protein fractions of blood, content of A, G immunoglobulins, M can be observed. The feeling of an itch at patients with a pemphigus, as a rule, does not happen. Sometimes numerous erosion cause severe pains which amplify during bandagings and at change of position of a body. Defeats at their localization in an oral cavity, on a red border of lips and genitals are especially painful.

Reasons Bubbly dermatitis:

Direct evidence of the autoimmune nature of a true pemphigus was obtained in the sixties when immunofluorescent methods showed presence of the circulating antibodies to epidermis antigens in blood serum of patients. The Immunoblotingovy analysis of antibodies to antigens of epidermis and vesical liquid served improvement of diagnosis and differential diagnosis of various forms of a pemphigus. In an organism of patients with a pemphigus find the circulating antibodies like IgG having affinity to intercellular substance of an acanthceous layer of epidermis and membrane antigens of acanthceous eiiteliotsit, the quantity of antibodies korrigirut with weight of a disease. Immune changes are the cornerstone of development of an acantolysis, active participation in this process belongs to also cellular cytotoxic reactions, disturbance of balance in kallikrein-kinin system, to participation of limfokinopodobny substances, eicosanoids, endoproteinases and their inhibitors. Apparently, the expression on a surface of acanthceous cells of complete (immune) antigen of a pemphigus which unlike incomplete (physiological) induces an afferent link of autoimmune reaction is the reason of disappearance of tolerance to membrane antigens of acanthceous epithelial cells. It is not excluded that it is connected with change of kernels of acanthceous cells. It is established that at a pemphigus in kernels of akantolitichesky cells the content of DNA is increased, and there is feedforward between increase in content of nuclear DNA and disease severity that, apparently, leads to change of an antigenic structure of these cells and development of autoantibodies against it like IgG. The important role in a pathogeny of a pemphigus belongs to changes of T - and V-lymphocytes and if responsibility for activity of pathological process lies on V-lymphocytes, then the quantity and a functional condition of T lymphocytes define emergence and the course of a disease.

Treatment Bubbly dermatitis:

With administration of corticosteroid hormones the forecast at a pemphigus became more favorable. Treatment begins with use of shock optimum doses of hormones (60-80 mg and more in days), the therapeutic effect further reached is supported by out-patient treatment and observation. Doses are established by the doctor individually for each patient.
Are recommended: Prednisonum in tablets on 0,005 g, Prednisolonum, dexamethasone in tablets on 0,00005 g and 0,001 g, триамсиналон in tablets on 0,004 g etc.
Duration of use of the maximum daily dose is defined by disease severity, age of the patient and portability of drug. On reaching therapeutic effect the daily dose is gradually and very slowly reduced. The maintenance therapy is continued for many years.
Damage of mucous membranes takes place more slowly, and under the influence of high doses of hormones. The patient's food is of great importance. Food has to be vitamin-rich, proteins. It is necessary to limit reception of table salt and carbohydrates. Treatment is combined with anabolic hormones (Nerobolum, retabolil etc.).
Outwardly apply various disinfectants. The complications coming at treatment by hormones can be divided into 3 groups: lungs (increase in arterial pressure, Icenco-Cushing syndrome, etc.); moderately severe (osteoporosis, defeat of cardiovascular system, digestive tract, pyoderma, diabetes etc.); heavy, leading to death of the patient (wet brain, acute cardiovascular insufficiency, profuse bleedings etc.).
Except corticosteroids, appoint immunodepressants a methotrexate, Azathioprinum etc.