True puzyrchatka – a chronic disease of the autoimmune nature which is characterized by emergence of bubbles on clinically healthy skin and mucous membranes. Features of a clinical current of a puzyrchatka allowed clinical physicians to distinguish the following forms of a disease: vulgar, eritematozny, vegetiruyushchy and listovidny. Puzyrchatka is diagnosed at detection of akantolitichesky cages in dab print and histologic identification of the bubbles located in epidermis. In treatment of a puzyrchatka the basic is course purpose of glucocorticosteroids, it is successfully combined with methods of extracorporal haemo correction: plazmoforezy, haemo sorption, krioaferezy.
True puzyrchatka – a chronic disease of the autoimmune nature which is characterized by emergence of bubbles on clinically healthy skin and mucous membranes.
Reasons of a puzyrchatka
The most probable violations of autoimmune processes therefore cages of an organism become antibodies for immune system are the reason of a puzyrchatka. Violation of anti-gene structure of cells of epidermis happens under the influence of external factors, in particular influence of retroviruses and aggressive conditions of the environment.
The damaging action on cells of epidermis and development of specific anti-genes leads to disruption of communication between cages therefore bubbles are formed. Risk factors at a puzyrchatka are not established, but at persons with hereditary predisposition the percent of incidence is higher.
Clinical manifestations of a puzyrchatka
Puzyrchatka has a long wavy current, and lack of adequate treatment leads to violation of the general condition of the patient. At a vulgar form of a puzyrchatka bubbles are localized on all body, have the different size and are filled with serous contents, at the same time a tire on bubbles sluggish and thin.
The vulgar puzyrchatka as a rule on mucous a mouth and a nose debuts, and therefore patients it is long and unsuccessfully receive therapy at stomatologists and otolaryngologists. At this stage of a puzyrchatka patients show complaints to pain during meal and at a conversation, a gipersalivation and a specific unpleasant smell from a mouth. Duration of this period from three months to one year then the puzyrchatka accepts widespread character and integuments are involved in inflammatory process.
Sometimes patients do not notice existence of bubbles because of their small size and a thin tire, bubbles are opened quickly, and therefore the main complaints of patients with a puzyrchatka on this period - it is painful erosion. Long and unsuccessful therapy of stomatitises before diagnose a puzyrchatka is carried out. Bubbles which are localized on skin tend to spontaneous opening with an exposure of an erozirovanny surface and from the remains of a tire which shrinks in crusts.
Erosion at a puzyrchatka bright pink, with a smooth glossy surface, differ from erosion at other diseases of tendency to peripheral growth and to generalization with formation of the extensive centers of defeat. If the puzyrchatka accepts such current, then the general condition of the patient worsens, develops intoxication, the secondary infection joins and without due treatment such patients die. At a vulgar puzyrchatka Nikolsky's syndrome positive in the center of defeat and sometimes on healthy skin - at insignificant mechanical influence occurs an otsloyka of the top layer of an epithelium.
The Eritematozny puzyrchatka differs from vulgar in the fact that at the beginning integuments are surprised; the eritematozny centers on a breast, a neck, a face and on a hairy part of the head have seboreyny character, have a clear boundary, the surface is covered with yellowish or brown crusts of various thickness. If to separate these crusts, then the erozirovanny surface is bared.
At an eritematozny puzyrchatka bubbles can be small, their tire flabby and sluggish, they are very quickly spontaneously opened therefore to diagnose a puzyrchatka extremely difficult. Nikolsky's symptom, as well as at an eritematozny puzyrchatka, can have several years the localized character, then at generalization of process gains lines vulgar. The Eritematozny puzyrchatka should be differentiated with a red volchanka and with seboreyny dermatitis.
The Listovidny puzyrchatka is clinically shown by eritemo-skvamozny rashes, thin-walled bubbles tend to appear on earlier affected areas, after opening of bubbles the bright red erozirovanny surface when which drying lamellar crusts are formed is bared. As at such form to a puzyrchatka bubbles arise also on crusts, the affected skin sometimes is covered with a massive layered crust at the expense of constant office of exudate. The sheet puzyrchatka affects skin, but defeats mucous are seldom or never observed, it quickly extends on all healthy skin and at the same time on skin there are bubbles, crusts and erosion which merging with each other, form an extensive wound surface. Nikolsky's symptom positive even on healthy skin; with accession of pathogenic microflora sepsis develops from what usually and there comes the death of the patient.
The Vegetiruyushchy puzyrchatka proceeds dobrokachestvenny, sometimes patients for many years are in satisfactory condition. Bubbles are localized around natural openings and in the field of skin folds. Being opened, bubbles bare erosion at the bottom of which soft vegetations with a fetid smell are formed; vegetations from above are covered with a serous or serous and purulent raid. On the periphery of educations there are pustula, and therefore the vegetiruyushchy puzyrchatka is required to be differentiated from a vegetiruyushchy chronic piodermiya. Nikolsky's syndrome positive only near the affected skin, but in terminal stages the vegetiruyushchy puzyrchatka is similar with vulgar in the clinical manifestations.
Diagnostics of all types of a puzyrchatka
Clinical manifestations especially at initial stages of a disease of a maloinformativna and therefore poll of the patient allows to avoid the wrong diagnosis. Laboratory researches allow to suspect a puzyrchatka, so in dabs prints at a cytologic research akantolitichesky cages are found. At a histologic research the vnutriepidermalny arrangement of bubbles is found.
Principles of treatment of a puzyrchatka
The hypoallergenic diet and exception of a diet of rough food, canned food, simple carbohydrates, salty products and other extractive substances are shown to patients with any forms of a puzyrchatka. If the oral cavity is surprised, then it is necessary to include cream soups and mucous porridges in a diet not to allow full refusal of food, food rich with protein, included in a diet, accelerate process of cell regeneration and an epitelization of open erosion.
All patients patients with a puzyrchatka have to be on dispensary observation at the dermatologist, the sparing operating mode, lack of physical activities and avoiding of insolation is recommended. Frequent change of next-to-skin and bed linen prevents accession of a secondary infection.
Purpose of glucocorticosteroids in high doses as differently the therapeutic effect will not be reached is shown at once, after knocking over of sharp manifestations of a puzyrchatka of a dosage of hormonal medicines gradually reduce to minimum effective. In treatment of a puzyrchatka apply methods of extracorporal haemo correction: haemo sorption, and membrane plasma exchange. As local treatment of a puzyrchatka use aniline dyes and nonaggressive antiseptic solutions.
The forecast of a puzyrchatka always adverse as in case of lack of adequate treatment the death of patients comes quickly enough from the joined complications. Long hormonal therapy in high doses increases risk of emergence of side effects, but at refusal of glucocorticosteroids the puzyrchatka begins to recur.
Puzyrchatka of newborns
Puzyrchatka of newborns – an acute, high-contagious infectious disease of skin, is clinically shown in a look which very quickly extend on an integument. Unlike a true puzyrchatka, the puzyrchatka of newborns has the bacterial nature and its activator is golden staphylococcus.
In pathogenesis of a puzyrchatka of newborns the important place is taken by reactivity of skin of just been born children which amplifies at patrimonial injuries, prematurity and at the wrong way of life of the pregnant woman. In response to action of bacterial factors on skin bubbles are formed and diagnose a puzyrchatka of newborns. In epidemiology of a puzyrchatka of newborns violation of hygienic norms in maternity hospitals, presence at personnel of the centers of a chronic infection, perhaps autoinfitsirovany a puzyrchatka lies if at the newborn purulent diseases of a navel develop.
Puzyrchatka of newborns arises in the first days of life of the child, but a disease perhaps and 1-2 weeks later. On clinically healthy or slightly eritematozny skin small intense thin-walled bubbles with serous contents appear. In several hours process generalizutsya, bubbles increase in sizes and are opened. On the place of bubbles there are painful erosion from the epidermis remains at the edges, erosion become covered by serous and purulent crusts. At a puzyrchatka of newborns at children intoxication symptoms, the increased temperature are observed, many of them refuse food.
In the absence of adequate treatment the puzyrchatka of newborns provokes inflammatory processes of internals: pneumonia, otitises, phlegmons. At weak and deeply premature children the septic form of a puzyrchatka at which lethality is rather high is possible.
Diagnose a puzyrchatka of newborns on the basis of visual survey, it is necessary to differentiate her with a syphilitic puzyrchatka which is display of congenital syphilis at which bubbles are localized on palms.
Antibitotikoterapiya allowed to lower considerably mortality percentage from a puzyrchatka of newborns whereas earlier more than a half of sick children died, the forecast of a puzyrchatka of newborns favorable at timely and adequate therapy. Locally apply aniline dyes and nonaggressive antiseptics.
Preventive measures are change bed and underwear, discharge from work of personnel with pustulous rashes, observation of pregnant women and timely local therapy at mothers of pustulous rash.