Pemphigus
Pemphigus is a chronic autoimmune disease characterized by the appearance of a special type of blisters on the surface of previously healthy skin and mucous membrane. Among the types of pemphigus can be distinguished: vulgar, vegetative, erythematous and leaf-like. Pemphigus can be diagnosed if acantholytic cells are detected, which are detected in a smear taken or as part of blisters in the epidermis itself (during histological examination). For the treatment of pemphigus, glucocorticosteroids are first used (a whole course of administration is prescribed). The latter always goes well with extracorporeal hemocorrection (plasmophoresis, cryoaferosis, hemosorption).
The content of the article:
Causes of pemphigus
Symptoms of pemphigus
Vulgar pemphigus
Pemphigus in adults
Erythematous type of pemphigus
Leaf – like type of pemphigus
Vegetative type of pemphigus
Diagnosis of pemphigus
Treatment of pemphigus
Pemphigus of newborns
Pemphigus
Causes of pemphigus
The most likely cause of pemphigus is considered to be violations of the processes of the autoimmune system, which is why the body’s cells present antibodies. Changes in the antigenic structure of the epidermis itself are observed due to the influence of external factors (for example, the effect of retroviruses or poor environmental conditions).
A negative effect on the epidermis and the production of antigens has a violation of the connection between cells, which is why bubbles appear later. As for the risk factors for the course of pemphigus, they have not yet been established, but it is known that people with a hereditary predisposition have a high percentage of morbidity.
Symptoms of pemphigus
Pemphigus is characterized by a long undulating flow. In the absence of proper treatment, there may be a violation of the general condition of the patient. In the case of a vulgar form of pemphigus, the bubbles themselves will be located over the entire surface of the body, representing formations of different sizes that are filled with serous fluid. The surface of such bubbles will be thin and sluggish.
Vulgar pemphigus
The first thing that occurs is pemphigus on the mucous membranes, so as a rule, the initial treatment will be incorrect — usually people are treated by a dentist or an otolaryngologist. At this stage of the disease, patients complain of sore throat during meals or even when talking. There is also hypersalivation and bad breath. The duration of such a symptomatic period ranges from three to four months to a year. After that, the pemphigus is characterized by its spread over the surface of the body, involving an increasing area of the skin.
Often patients may not notice the presence of bubbles due to their small size, as well as a thin film on top. Usually, blisters can open quickly, so patients’ complaints are complaints about painful erosion. Long-term and almost always unsuccessful treatment of stomatitis is often carried out. Symptoms of pemphigus are primarily blisters localized on the skin. They are distinguished by the possibility of self-opening, while exposing the eroded surface with the remains of the tire, which often dries out and takes the form of a crust.
Pemphigus in adults
Pemphigus in adults may have a bright pink color, a smooth glossy surface. Pemphigus should not be confused with erosion. Pemphigus differs from the latter in its tendency to peripheral growth and the possibility of generalization with the further formation of extensive lesions. If the pemphigus takes such a course, the general condition of the patient almost always worsens, the development of intoxication begins, it is also possible to attach a secondary infection. Without proper treatment, patients may die.
In the case of vulgar pemphigus, Nikolsky’s syndrome will be positive in the lesion. On healthy skin, in the case of even minor mechanical action, there may be detachment of the upper layer of the epithelium.
Erythematous type of pemphigus
The erythematous type of pemphigus will differ from the vulgar type by the localization of skin lesions, the presence of erythematous foci on the chest, neck and face, as well as on the scalp. The latter is seborrheic in nature. Erythematous pemphigus is characterized by clear boundaries, its surface is covered with a crust of yellow or brown color. If such crusts are separated from the main surface, then the entire eroded surface will be exposed.
In the case of erythematous pemphigus, the blisters are usually small in size, the crust is flabby and sluggish in appearance, they often open themselves, so it is very difficult to diagnose pemphigus. Nikolsky’s symptom can have a localized character for a long time, and in the case of generalization of the process it becomes similar to a vulgar appearance.
The erythematous type of pemphigus should be differentiated from the seborrheic type of dermatitis and with lupus erythematosus.
Leaf – like type of pemphigus
The leaf-like type of pemphigus, according to its symptoms, represents erythema-squamous type rashes, blisters have thin walls and often occur on already once affected areas. After opening the bubbles, the eroded surface becomes bright red. When the surface dries, lamellar crusts appear. Since with this form of pemphigus, bubbles form on the crusts, the affected area of the skin can be covered with a massive layer of crust due to the abundant separation of exudate.
The leafy type of pemphigus captures the entire skin, but the mucous membranes are also rarely involved. Leaf pemphigus quickly covers the entire skin, on which bubbles, erosions and fresh crusts immediately appear. Combining with each other, the damaged areas form a large wound surface. Nikolsky’s syndrome will be positive even on a healthy area of the skin. In the case of the addition of pathogenic microflora, sepsis will begin to develop, which is why a person’s death occurs.
Vegetative type of pemphigus
The vegetative type of pemphigus proceeds benign. Patients can feel well for many years. The blisters are located around the holes, as well as in the area of skin folds. When the bubbles open, they expose erosions, at the bottom of which soft vegetation with a fetid smell appears. Such vegetation is covered with serous-purulent or simply serous fluid. According to the contour of neoplasms, pustules can be considered, therefore, vegetating pemphigus should be distinguished from the chronic form of pyoderma. Nikolsky syndrome will be positive only near the affected areas of the skin, however, in the terminal stages, vegetative pemphigus is similar to vulgar in its clinical manifestations.
Diagnosis of pemphigus
The clinic of this disease, especially in the early stages of the disease, will be uninformative, so the patient’s survey will help to exclude an erroneous diagnosis. Laboratory tests will help to identify pemphigus by detecting acantholytic cells during cytological examination. During histological studies, the intraepidermal location of the bubbles themselves will be detected.
Treatment of pemphigus
Treatment of pemphigus, first of all, consists in the exclusion of coarse food, simple carbohydrates, canned food, salted and salted foods from the usual diet. In case of damage to the oral cavity, it is necessary to add soups and rare porridges to the diet, so as not to exclude from the diet a complete rejection of food. It is known that products rich in protein content accelerate the process of cell regeneration and influence the process of epithelization of open erosions.
All patients suffering from pemphigus are registered with a dermatologist at the dispensary. For such people, a light work regime is shown, cessation of physical exertion, avoidance of insolation. Frequent change of bed and underwear will help prevent the addition of secondary infection.
Treatment of pemphigus also consists in the use of glucocorticosteroids, while using them in large doses. Otherwise, positive dynamics in treatment will not be achieved. After the relief of acute symptoms of pemphigus, the dosage of the drugs used is reduced to the minimum level. Extracorporeal hemocorrection is also used for treatment, which includes cryoapheresis, membrane plasmapheresis and hemosorption.
For local treatment of pemphigus, non-aggressive antiseptic solutions and aniline dyes are used. The prognosis of pemphigus is almost always unfavorable, because in the absence of adequate treatment, a person’s death can occur quite quickly due to complications. Prolonged hormone therapy significantly increases the risk of side effects, but if you refuse to take glucocorticosteroids, the pemphigus will recur.
Pemphigus of newborns
Pemphigus in newborns is an acute infectious skin disease, which is clinically manifested in the form of pustules that quickly spread throughout the skin.
Pemphigus in newborns is often bacterial in nature. Its causative agent is Staphylococcus aureus. Speaking about the pathogenesis of pemphigus among newborns, a significant place is occupied by the reaction of the skin of children. The reaction of the skin will increase in case of birth trauma or prematurity, as well as the wrong lifestyle of the pregnant woman herself. Blisters will form on the baby’s skin due to the effects of bacterial factors.
The epidemiology of pemphigus in newborns indicates a violation of hygiene in the maternity hospital, the presence of chronic infections in maternity hospital staff, the possible occurrence of auto-infected pemphigus (if the newborn develops purulent types of navel diseases).
Pemphigus in newborns is formed in the first days of life, but the development of the disease is possible after one or two weeks. On healthy skin, small bubbles with thin walls appear, having serous contents. After a few hours, the process will generalize, the bubbles will increase in size and open. Painful erosions with the remaining particles of the epidermis located at the edges will remain in place of the bubbles. Such erosions will be covered with a serous-purulent crust. In the case of pemphigus, intoxication, fever and lack of appetite will be present in newborns.
If the pemphigus is not cured in the early stages, the newborn will develop inflammatory processes of internal organs (phlegmons, otitis media, pneumonia). In weak newborns or premature infants, the septic form of pemphigus is not excluded. With the latter, the lethal outcome is very high.
It is possible to diagnose pemphigus in newborns on the basis of a visual examination. It is necessary to distinguish pemphigus in newborns from the syphilistic form of pemphigus, which is a symptom of congenital syphilis. With the latter, the bubbles are located on the palms.
Antibiotic therapy can reduce the percentage of deaths from pemphigus among newborns. With timely treatment of pemphigus among newborns, the favorable outcome of the disease is significantly higher than with other types. Doctors may also prescribe the use of aniline dyes and various kinds of non-aggressive antiseptics.
Prevention of pemphigus is considered to be a change of underwear and bed linen, isolation of those who have pustular rashes on the skin, as well as proper monitoring of pregnant women and timely treatment among mothers with pustular rashes.
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