Pemphigus of a newborn is a very rare and dangerous disease, which is based on the mechanisms of an autoimmune attack on the skin integument. Under the influence of unknown factors, the immune system produces antibodies to substances that ensure the integrity of the skin. They act on epithelial cells, as a result of which ulcers appear on the skin, which become infected with streptococcus or staphylococcus.
Description
The official name of the disease is pemphigus, the symptoms of which are the presence of destructive antibodies in the blood.
Pemphigus can affect people of any age and gender. The disease is very dangerous for newborns in whom local immunity has not yet been formed. Pemphigus is prone to progression, and with extensive lesions, the body quickly loses fluid. The risk of infection is extremely high, as bacteria and fungi can lead to the development of sepsis.
Varieties
To speciespemphigus of newborns (in the photo you can see how the manifestations of the disease look) include:
- Pemphigus vulgaris.
- Vegetative pemphigus.
- Leaf-shaped.
- Erythematous.
- Seborrheic.
Vulgar pemphigus is considered the most common. In this case, vesicles appear on the skin, which are filled with serous fluid. They break easily and heal quickly. The first blisters appear most often around the mouth and lips.
These blisters over time appear more and more extensively and spread throughout the body, and after their breakthroughs remain pink eczema. If this disease is not treated, the child may die within six months.
When the vegetative form begins the formation of scattered single bubbles. After they burst, gray papillomas (vegetations) remain on the skin, prone to growth.
Leaf shape
The leaf-shaped form of pemphigus of the newborn is characterized by the formation of crusts that occur after the rupture of the blisters. The peculiarity of the disease is that it develops rapidly. Crusts are formed very large, after which they begin to peel off in large pieces like leaves. It is possible to attach erythematous pemphigus to this form. There is no significant difference between them, since the symptoms and prognosis are similar.
Seborrheic form
Seborrheic form begins to develop from the scalp, from the face. The disease progressesvery slowly, blisters begin to appear on the back, limbs, abdomen. When removing the crust, weeping eczema is formed.
There are other forms of pemphigus:
- viral;
- syphilitic.
Viral pemphigus of the newborn is caused by the Coxsackie virus, as well as 71 species of enteroviruses. The disease can take on grandiose proportions and become epidemic. In children, it appears mainly on the palms and feet, although blisters may appear on the genitals.
Syphilitic pemphigus of newborns develops as a result of intrauterine infection with syphilis. Children may develop pus-filled blisters that break open fairly quickly, leaving pinkish eczema.
The paratumoral pemphigus has the most striking clinical signs. As a rule, it often accompanies diseases such as leukemia or lymphoma, and may indicate the onset of a malignant process.
Let's look at the main causes of pemphigus in newborns.
Causes of pathology in babies
The onset of the disease coincides with the production of autoimmune antibodies to desmoglein proteins, which are the basis for connecting epidermal cells. Proteins are broken down and the integrity of the skin suffers, and bacteria, when they get on the affected skin, cause the formation of blisters.
Factors that can provoke such a pathological process are still unknown to science. The main reason is genetics and hereditary predisposition to such an autoimmune process. Disturbances in the work of 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.
Provoking factors
The following are also indicated as possible factors for the occurrence of pathology:
- Taking thiol group medicines.
- Burns.
- Herpesviruses.
- Contact with pesticides.
- Strong stress.
Pemphigus in the early stages does not cause any concern. Children feel well and apart from a few vesicles on the skin, no other changes are observed. Deterioration of the condition develops according to the increase in the area of the affected areas of the skin. The area of the lesion grows when a bacterial infection enters the skin, and the development of local suppurations and large inflammatory foci begins. This disease is characterized by the absence of tissue epithelialization after rupture of the bladder. Erosions gradually expand and merge with each other. How is neonatal pemphigus diagnosed?
Diagnosis
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 child's skin nearbywith the bubble and away from it, presses on the blister. The test is positive if there are signs of skin exfoliation:
- When pressed, the liquid spreads into the layers of the skin.
- If you press on the skin over the blister, it peels off like a ribbon.
- In a he althy area, a mixture of the upper layers of the epidermis is noticed.
An analysis is also prescribed for the content of antibodies to desmoglein proteins in the blood. If such antibodies are found, then this indicates the presence of pemphigus. Sometimes fluid samples are taken from the blisters and additional general or cytological studies are ordered.
Treatment of pemphigus in newborns
The main thing in the treatment of pathology is to prevent the formation of new blisters and 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 glucocorticosteroids, which are administered in high doses. This makes it possible to reduce the intensity of the formation of new bubbles and start the process of erosion recovery. This therapy is carried out for about two weeks, after which maintenance hormonal treatment with the same drug in small doses is necessary.
Prednisolone
The most effective drug in the treatment of pemphigus is Prednisolone, which is administered in large doses, which are systematically reduced. Unfortunately, for most children, such treatment lasts for life, and Prednisolone is injected everyday.
Along with hormones, the child is prescribed calcium, vitamin D, immunosuppressive drugs that suppress the activity of the immune system. These medications 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 therapy for pemphigus will be lifelong. In rare cases - with minor breaks between relapses.
Baby care
The he alth of children diagnosed with staphylococcal pemphigus of the newborn requires special attention and daily care. The life expectancy of such a child depends on this. It is very important to make sure that he takes all the drugs prescribed by the doctors.
Parents will have to learn how to give shots, because it will have to be done every day. It is also necessary to treat blisters on the skin with aniline dyes ("Fukortsin", Zelenka), which are highly effective against microbes. Corticosteroid ointments are recommended for erosions and crusts. As a rule, "Celestoderm", "Garamycin" and "Gyoksizon" are prescribed.
When signs of infection appear - pus, swelling - it is necessary to use antibacterial ointments during treatment -"Baneocin" or "Levomekol". If large areas are affected, it is best to protect them with a sterile dressing.
Anti-inflammatory drugs
When complaining of pain, it is sometimes allowed to give the child anti-inflammatory drugs Ibuprofen or Nurofen. If anesthesia fails, 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.
