Pemphigus is a chronic autoimmune disease characterized by the appearance of blisters on clinically healthy skin and mucous membranes.Features of the clinical course allowed clinicians to identify the following forms of the disease: vulgar, erythematous, vegetative and leaf-like. Disease is diagnosed when acantholytic cells are detected in a smear print and histological detection of blisters located inside the epidermis. In the treatment of pemphigus, the main course is the appointment of glucocorticosteroids, it is successfully combined with methods of extracorporeal hemocorrection: plasmophoresis, hemosorption, cryoapheresis.
Causes of pemphigus
The most likely cause of pemphigus are disorders of autoimmune processes, as a result of which the body’s cells become antibodies for the immune system. Violation of the antigenic structure of epidermal cells occurs under the influence of external factors, in particular the effects of retroviruses and aggressive environmental conditions.
The damaging effect on the cells of the epidermis and the production of specific antigens leads to a violation of the connection between the cells, as a result of which bubbles are formed. Risk factors have not been established, but in people with a hereditary predisposition, the incidence rate is higher.
Pemphigus has a long undulating course, and the lack of adequate treatment leads to a violation of the general condition of the patient. With a vulgar form, the bubbles are localized throughout the body, have different sizes and are filled with serous contents, while the tire on the bubbles is sluggish and thin.
Vulgar pemphigus usually debuts on the mucous membrane of the mouth and nose, and therefore patients receive therapy from dentists and otolaryngologists for a long time and unsuccessfully. At this stage of pemphigus, patients complain of pain during meals and during conversation, hypersalivation and specific bad breath. The duration of this period is from three months to a year, after which the pemphigus becomes widespread and the skin is involved in the inflammatory process.
Sometimes patients do not notice the presence of bubbles due to their small size and thin tire, the bubbles open quickly, and therefore the main complaints of patients with pemphigus at this period are painful erosions. Prolonged and unsuccessful therapy of stomatitis is carried out before pemphigus is diagnosed. Blisters that are localized on the skin tend to spontaneously open with the exposure of the eroded surface and with the remnants of the tire, which shrivels into crusts.
Erosions are bright pink, with a smooth glossy surface, differ from erosions in other diseases by a tendency to peripheral growth and generalization with the formation of extensive lesions. If the pemphigus takes such a course, then the general condition of the patient worsens, intoxication develops, a secondary infection joins and without proper treatment such patients die. With vulgar pemphigus, Nikolsky syndrome is positive in the lesion and sometimes on healthy skin – with minor mechanical action, the upper layer of the epithelium is detached.
Erythematous pemphigus differs from vulgar in that the skin is affected at the beginning; erythematous foci on the chest, neck, face and scalp are seborrheic in nature, have clear boundaries, the surface is covered with yellowish or brown crusts of varying thickness. If these crusts are separated, the eroded surface is exposed.
With erythematous pemphigus, the bubbles may be small, their tire is flabby and sluggish, they open spontaneously very quickly, therefore it is extremely difficult to diagnose pemphigus. Nikolsky’s symptom, as with erythematous pemphigus, can be localized for several years, then, with the generalization of the process, it acquires vulgar features. Erythematous pemphigus should be differentiated from lupus erythematosus and seborrheic dermatitis.
Leaf-shaped form is clinically manifested by erythema-squamous rashes, thin-walled blisters tend to appear on previously affected areas, after opening the blisters, a bright red eroded surface is exposed, when drying, lamellar crusts are formed. Since with this form of pemphigus, bubbles also appear on the crusts, the affected skin is sometimes covered with a massive layered crust due to the constant separation of exudate. Leaf pemphigus affects the skin, but in very rare cases mucosal lesions are observed, it quickly spreads throughout healthy skin and at the same time there are blisters, crusts and erosions on the skin, which merge with each other to form an extensive wound surface. Nikolsky’s symptom is positive even on healthy skin; with the addition of pathogenic microflora, sepsis develops, from which the patient usually dies.
Vegetative form is more benign, sometimes patients are in a satisfactory condition for many years. Blisters are localized around natural openings and in the area of skin folds. Opening, the bubbles expose erosions, at the bottom of which soft vegetation with a fetid odor is formed; vegetation is covered with a serous or serous-purulent coating on top. There are pustules on the periphery of the formations, and therefore vegetative pemphigus needs to be differentiated from vegetative chronic pyoderma. Nikolsky syndrome is positive only near the affected skin, but in the terminal stages, vegetative form is similar to vulgar in its clinical manifestations.
Clinical manifestations, especially at the initial stages of the disease, are uninformative, and therefore the patient’s survey avoids an erroneous diagnosis. Laboratory studies make it possible to suspect pemphigus, so acantholytic cells are detected in smears during cytological examination. Histological examination reveals the intraepidermal location of the bubbles.
A hypoallergenic diet and exclusion from the diet of coarse food, canned food, simple carbohydrates, salty foods and other extractive substances are indicated for patients with any forms of pemphigus. If the oral cavity is affected, then it is necessary to include mashed soups and mucous porridges in the diet in order to prevent complete rejection of food, protein-rich foods included in the diet accelerate the process of cell regeneration and epithelization of open erosions.
All patients should be monitored by a dermatologist at a dispensary, a gentle mode of operation is recommended, lack of physical exertion and avoidance of insolation. Frequent change of underwear and bed linen prevents the addition of secondary infection.
The appointment of glucocorticosteroids is shown immediately in high doses, since otherwise no therapeutic effect will be achieved, after the relief of acute manifestations of pemphigus, the dosage of hormonal drugs is gradually reduced to the minimum effective. In the treatment of pemphigus, methods of extracorporeal hemocorrection are used: hemosorption, cryoapheresis and membrane plasmapheresis. Aniline dyes and non-aggressive antiseptic solutions are used as local treatment of pemphigus.
The prognosis of pemphigus is always unfavorable, since in the absence of adequate treatment, the death of patients occurs quite quickly from the associated complications. Long-term hormone therapy in high doses increases the risk of side effects, but with the rejection of glucocorticosteroids, the pemphigus begins to relapse.
Pemphigus of newborns
Pemphigus of newborns is an acute, highly contagious infectious skin disease, clinically manifested in the form of pustules that spread very quickly through the skin. Unlike true pemphigus, the pemphigus of newborns has a bacterial nature and its causative agent is Staphylococcus aureus.
In the pathogenesis of the pemphigus of newborns, an important place is occupied by the reactivity of the skin of newly born children, which increases with birth injuries, prematurity and with an incorrect lifestyle of a pregnant woman. In response to the action of bacterial factors, blisters form on the skin and the pemphigus of newborns is diagnosed. The epidemiology of the pemphigus of newborns is a violation of hygiene standards in maternity hospitals, the presence of foci of chronic infection in the staff, autoinfection with pemphigus is possible if the newborn develops purulent diseases of the navel.
Disease of newborns occurs in the first days of a child’s life, but the disease is possible 1-2 weeks later. On clinically healthy or slightly erythematous skin, small tense thin-walled bubbles with serous contents appear. After a few hours, the process is generalized, the bubbles increase in size and open. In place of blisters, painful erosions remain with remnants of the epidermis at the edges, erosions are covered with serous-purulent crusts. With pemphigus of newborns, children have symptoms of intoxication, fever, many of them refuse food.
In the absence of adequate treatment, the pemphigus of newborns provokes inflammatory processes of internal organs: pneumonia, otitis media, phlegmon. In weak and deeply premature infants, a septic form of pemphigus is possible, the mortality rate of which is quite high.
The pemphigus of newborns is diagnosed on the basis of visual examination, it is necessary to differentiate it with syphilitic pemphigus, which is a manifestation of congenital syphilis, in which the bubbles are localized on the palms.
Antibiotic therapy has significantly reduced the percentage of mortality from pemphigus of newborns, whereas previously more than half of sick children died, the prognosis of pemphigus of newborns is favorable with timely and adequate therapy. Aniline dyes and non-aggressive antiseptics are applied topically.
Preventive measures are the change of bed and underwear, the removal from work of staff with pustular rashes, monitoring of pregnant women and timely local therapy for mothers of pustular rash.