Pemphigus is a very rare and dangerous disease, which is based on the mechanisms of autoimmune attack on the skin . Under the influence of unknown factors, immunity produces antibodies to substances that ensure skin integrity. They act on epithelial cells, as a result of which ulcers appear on the skin that become infected with streptococcus or staphylococcus.
Description
The official name of the disease is pemphigus, the signs of which are the presence of destructive antibodies in the blood.
Pemphigus can affect people of any age and gender. A disease is very dangerous for newborns in whom local immunity is not yet formed. Pemphigus is prone to progression, and with extensive lesions, the body quickly loses fluid. The risk of infection is extremely high, since bacteria and fungi can lead to the development of sepsis.
Varieties
Types of pemphigus in newborns (in the photo you can see how the manifestations of the disease look) include:
- Pemphigus vulgaris (vulgar).
- Vegetative pemphigus.
- Leaf-shaped.
- Erythematous.
- Seborrheic.
Pemphigus vulgaris is considered the most common. In this case, bubbles appear on the skin that are filled with serous fluid. They burst easily and heal soon. The first blisters appear most often in the mouth and lips.
Over time, these blisters appear more and more extensively and spread throughout the body, and pink eczema remains after their breakthroughs. If this disease is not treated, a child may die within six months.
With the vegetative form, the formation of scattered single bubbles begins. After they burst, gray papillomas (vegetation) remain on the skin, prone to overgrowth.
Leaf shape
The leaf-shaped form of the pemphigus of the newborn is characterized by the formation of crusts that occur after the bursting of the bubbles. A feature of the disease is that it develops rapidly. The crusts are formed very large, after which they begin to exfoliate in large pieces like leaves. The erythematous pemphigus is possible to this form. There is no significant difference between them, since the symptoms and prognosis are similar.
Seborrheic form
The seborrheic form begins to develop from the scalp, from the face. The disease progresses very slowly, blisters begin to appear on the back, limbs, abdomen. When removing the crust, weeping eczema forms.
There are other forms of pemphigus:
Viral pemphigus of the newborn is caused by the Coxsackie virus, as well as 71 species of enteroviruses. The disease can take on a grandiose scale and become epidemic. In children, it manifests itself mainly on the palms and feet, although the appearance of vesicles on the genitals is possible.
Syphilitic pemphigus in newborns develops as a result of intrauterine infection with syphilis. Children can become covered with blisters with purulent contents, which open quite quickly, leaving pinkish eczema.
The most striking clinical signs are paraneoplastic pemphigus. It, as a rule, often accompanies diseases such as leukemia or lymphoma, and may indicate the onset of a malignant process.
Consider the main causes of pemphigus in newborns.
Causes of pathology in infants
The onset of the disease coincides with the development of autoimmune antibodies to desmogleins proteins, which are the basis for the connection of epidermal cells. Proteins are destroyed, and the integrity of the skin suffers, and bacteria, falling on the affected skin, cause the formation of bubbles.
Factors that can provoke such a pathological process are still unknown to science. The main reason is considered to be genetics and a hereditary predisposition to such an autoimmune process. Disorders in the central nervous system are also considered as a possible factor provoking the development of pemphigus. Infections, viruses or other pathogens can also trigger the onset of the disease.
Provocative factors
The following are also indicated as possible factors for the occurrence of pathology:
- Taking medications of the thiol group.
- Burns.
- Herpes viruses.
- Contact with pesticides.
- Severe stress.
Pemphigus in the early stages does not cause any concern. Children feel well and, in addition to several bubbles on the skin, no other changes are observed. Deterioration develops according to an increase in the area of affected skin. The area of the lesion grows when a bacterial infection enters the skin, and the development of local suppuration and large inflammatory foci begins. This disease is characterized by the absence of tissue epithelization after rupture of the bladder. Erosions gradually expand and merge with each other. How is the diagnosis of pemphigus newborns?
Diagnostics
The disease is very rare, so pediatricians often find it difficult to determine the diagnosis. First of all, it is recommended to distinguish the disease from other dermatological pathologies that may be accompanied by similar skin manifestations.
For this, the Nikolsky test is used. The doctor rubs the skin of the child near the bubble and away from it, presses the blister. The test is positive if there are signs of skin exfoliation:
- When pressed, the fluid spreads into the layers of the skin.
- If you press on the skin over the blister, it exfoliates in the form of a tape.
- On a healthy site, mixing of the upper layers of the epidermis is noticed.
An analysis is also prescribed for blood levels of antibodies to desmoglein proteins. If such antibodies are detected, then this indicates the presence of pemphigus. Sometimes fluid samples are taken from the blisters and additional general or cytological studies are prescribed.
Pemphigus treatment of newborns
The main thing in the treatment of pathology is to prevent the formation of new blisters and to achieve healing of existing ones. The main drugs in the treatment of the pathological process are glucocorticosteroids. Regardless of the age of the child, he is prescribed a course of systemic glucocorticosteroid drugs, which are administered in high doses. This allows you to reduce the intensity of the formation of new bubbles and start the process of restoring erosion. This therapy is carried out for about two weeks, after which supportive hormonal treatment with the same drug in small doses is necessary.
Prednisolone
The most effective medication for the treatment of pemphigus is Prednisolone, which is administered in large doses, which are systematically reduced. Unfortunately, for most children, this treatment lasts a lifetime, and Prednisolone is injected every day.
Along with hormones, the child is prescribed calcium preparations, vitamin D, immunosuppressive drugs that inhibit the activity of immunity. These medicines include:
- Azathioprine
- Cyclophosphamide;
- Mielosan
- "Cyclophosphamide."
In the early stages of therapy, the child is recommended procedures to cleanse the blood of aggressive antibodies. These include hemodialysis and plasmapheresis. To reduce the risk of infection, a high-quality treatment of the skin with ointments with antiseptics and corticosteroids is prescribed.
It should be clearly understood that pemphigus therapy will be lifelong. In rare cases, with insignificant intervals between relapses.
Baby care
The health of children with a diagnosis of staphylococcal pemphigus neonates requires special attention and daily care. The life expectancy of such a child depends on this. It is very important to ensure that he takes all medications prescribed by doctors.
Parents will have to learn how to inject injections, as they will have to do this every day. It is also necessary to treat blisters on the skin with aniline dyes (Fukortsin, Zelenka), which are highly effective against microbes. On erosion and crusts, it is recommended to apply corticosteroid ointments. As a rule, Celestoderm, Garamycin and Hyoxyson are prescribed.
If there are signs of infection - pus, swelling - it is necessary to use antibacterial ointments - "Baneocin" or "Levomekol" when processing. If large areas are affected, it is best to protect them with a sterile dressing.
Anti-inflammatory drugs
In case of complaints of pain, it is sometimes allowed to give the child anti-inflammatory drugs "Ibuprofen" or "Nurofen." If it is not possible to anesthetize, you need to consult a doctor who will tell you which analgesics are allowed for use in childhood. Baths in which you can add a solution of "Chlorhexidine" will benefit. It is necessary to give the child vitamin and mineral preparations with folic acid, vitamin E, etc.