A rare skin ailment known as pemphigus can strike children of any age, including newborns and infants. It manifests as tiny blisters on the skin, which can cause the child pain and discomfort. Even though this condition is rare, it’s crucial for parents to identify the symptoms as soon as possible to guarantee appropriate care.
Although the precise cause of pediatric pemphigus is not always known, it is believed to be related to the body’s immune system attacking the skin. Because these blisters can develop suddenly and be mistaken for other skin conditions, it’s critical to receive a proper diagnosis from a medical professional.
Prompt intervention can aid in symptom management and avoid complications. It’s wise to consult a doctor if your child experiences skin irritation or blisters that don’t seem to be related to anything. Maintaining your child’s comfort and health can be greatly improved by being aware of pemphigus and knowing what measures to take.
What is it?
Pemphigus is a fairly rare and dangerous disease. It is based on poorly understood mechanisms of an autoimmune attack on the skin. Under the influence of certain factors that are not yet obvious to science, the immune system begins to produce antibodies to substances that ensure the integrity of the skin. They actually "glue" the epithelial cells together. If these "adhesive" substances are destroyed, ulcers appear on the skin and mucous membranes, which are regularly infected with bacteria like streptococcus or Staphylococcus aureus. A more official name for the disease is pemphigus. Until the middle of the 20th century, pemphigus was any disease in which blisters or vesicles appeared on the skin. However, the main distinguishing features of pemphigus were then formulated – the presence of those same destructive antibodies in the blood. And diseases associated with the appearance of blisters are now divided into true pemphigus – pemphigus and other pemphigus, for which this name has been preserved mainly only among the people and out of habit.
A person with pemphigus can be any age or gender. Because they have virtually no developed local immunity, infants are most at risk. However, older children are also at serious risk from the disease. First of all, pemphigus usually gets worse. Second, a child loses fluid and proteins quickly when they have large skin lesions. Thirdly, there is a very high risk of infection; sepsis can be caused by both bacteria and fungi.
The number of children who contract pemphigus each year is unknown to pediatricians; however, the number of adult cases is estimated to be two per million people annually. The disease is particularly dangerous in warm-weathered nations and regions.
Types
- common (vulgar);
- vegetative;
- leafy;
- erythematous;
- seborrheic.
The most prevalent form of the illness is the vulgar kind. Blisters filled with serous fluid appear on the skin, which appears to be intact. They mend quickly and burst rather easily. The nasolabial triangle, which is located near the mouth, is where blisters typically first develop on the lips’ mucous membrane.
These bubbles, which initially might seem innocuous, eventually become larger and more frequent, spreading throughout the body. When they burst, pink eczema persists. A deadly outcome is possible if the child does not start receiving proper treatment within six months to two years. Similar to the typical form, the vegetative form of the disease starts off with the appearance of sporadic, single blisters. But after they burst, the child’s skin is left with grayish papillomas, or vegetation, that tend to grow instead of pink eczema.
The disease’s foliaceous form gets its name from the formation of crusts that follow the characteristic blisters that break. The disease is unique in that it progresses quickly; unlike regular pemphigus, there are no months during which the illness progresses slowly. Large crusts that peel off in chunks resembling leaves are present. Oftentimes, erythematous pemphigus joins this form. Doctors generally do not distinguish much between them because of how similar the symptoms and prognosis are.
The face and scalp are where the disease’s seborrheic form starts. Tiny bubbles soon develop into a crust that is yellow in color (much like seborrhea, which is how the form got its name). Blisters gradually start to appear on the back, abdomen, and limbs as the disease advances. Weeping eczema shows through when the yellowish-brown crust is lifted.
There are more prevalent types of pemphigus. They "fell out" of the general classification, though, once pemphigus was identified. However, they cannot be disregarded as long as they are referred to as pemphigus. These are the following:
- viral infectious pemphigus;
- syphilitic congenital pemphigus.
Certain pathogens, including enteroviruses of 71 subspecies and the Coxsackie virus (a very specific subspecies), are the cause of viral pemphigus, also known as infectious pemphigus. Enterovirus disease has the potential to grow enormously and spread rapidly. The viral form of the disease primarily affects the feet and palms in children, though blisters on the genitalia and buttocks are also possible.
An external sign of a syphilis intrauterine infection is syphilitic pemphigus. Blisters with serous contents may appear in children with congenital syphilis a few days after birth. Eczema that is weeping pink will result from the blisters popping quickly. Since the last two forms are merely symptoms of an underlying disease, medical professionals frequently refer to them as "symptoms" in these cases.
The attending physician, who will start treating the underlying disease first, will find this to be a much easier task. This is exactly the challenging part of true pemphigus, as the actual cause is frequently unknown.
Ppatumoral pemphigus, which is a true clinical sign, is the most noticeable. It coexists with serious illnesses like lymphoma and leukemia in 60% of cases. Occasionally, the development of distinctive skin symptoms both precedes and signals the beginning of a malignant tumor.
Causes
As was previously mentioned, the process of autoimmune aggressive antibodies to desmoglein proteins developing also occurs at the same time as the disease onset. These proteins serve as the fundamental "gluing" agent that maintains the adhesion of epidermal cells to one another. Skin integrity suffers as a result of the destruction of proteins. Additionally, blisters appear when bacteria—of which there are many—get into the exfoliated skin.
In medicine, the exact causes of such a pathological process are still unknown. The primary cause is still thought to be a genetic component, or a propensity for the development of this kind of autoimmune process. In addition, diseases of the central nervous system are taken very seriously as potential causes of pemphigus. The beginning of the disease may also be caused, in theory, by an infection, virus, or other pathogen; however, science is still unsure of the specific type of virus that could cause this. There is a correlation between endogenous bacterial infection and the development of pemphigus.
The Russian Ministry of Health’s 2016 clinical recommendations also list the following as potential causes of an insufficient immune response:
- taking thiol group drugs (penicillamine, captopril, cephalosporin antibiotics, immunomodulators);
- burns;
- herpes viruses types 1, 2 and 8;
- physical contact with pesticides;
- severe stress experienced by the child.
It sounds strange to hear the Ministry of Health officially state that certain foods high in tannin (like leeks, mango, raspberries, rosemary, vanillin, cherries, ginger, tea, and even common garlic) can also cause pemphigus.
Finding the true cause of the illness is not always feasible. Although the existence of aggressive antibodies in the body is verified in a lab, it is extremely uncommon to determine the reason behind their appearance.
Symptoms
Early on, pemphigus might not be a cause for concern. A few tiny blisters on the skin are the only changes, and the child feels fine overall. The area of the skin that is affected increases, and the condition gradually worsens.
The blisters exhibit what is known as "lethargy," opening with ease, and appearing to droop slightly on the skin covering the liquid inside. The oral cavity, specifically the mucous membranes or the area surrounding the lips, is where pemphigus first appears as rashes. It hurts a lot to get blisters.
Dense crusts or persistent wet eczema develop in the blisters’ place once they rupture. The afflicted region gets bigger, bacterial infections, localized purpura, inflammatory foci, erosions, and eczema of even greater extent follow. The absence of tissue epithelialization following bladder rupture is a characteristic of this disease. The erosions spread out and blend together over time.
Every variation of the illness has symptoms that appear in waves. Additionally, if you ignore them, the symptoms will worsen and include nausea, headaches, and intoxication. The patient may pass away from pemphigus.
Pemphigus is a rare but serious skin condition that affects children, infants, and newborns. It is characterized by the immune system attacking the skin by mistake, resulting in painful blisters and sores. To control the symptoms and avoid complications, early detection and treatment are essential. Parents can seek prompt medical attention by being aware of the warning signs, which include blisters that break easily and heal slowly. Medication is frequently used in treatment to lower inflammation and regulate the immune system.
Diagnostics
Occasionally, pediatricians face legitimate challenges in diagnosing the disease due to its rarity. It is first advised to distinguish this condition from other dermatological conditions that also cause blisters to appear, so as to avoid any confusion.
A technique known as Nikolsky’s test is applied in this case. The child’s skin is gently rubbed both close to and away from the blister by the doctor, who also lightly presses the blister with a fingertip. If symptoms of skin delamination manifest, the test is deemed positive:
- when pressed, the serous fluid spreads into the adjacent layers of the skin;
- if you gently pull the skin over the blister, it easily peels off in the form of a ribbon, like after a sunburn;
- when rubbing on a healthy area of the skin, a shift in the upper layer of the epidermis is noticeable.
It is always recommended to get a blood test to check for the presence of antibodies to desmoglein proteins. Their existence signifies the progression of pemphigus. In certain instances, the physician may extract serous fluid samples from the blisters and the erosion’s base in addition to recommending further cytological and general investigations (blood, urine).
At times, a chest X-ray and referrals to relevant specialists such as a cardiologist, nephrologist, or infectious disease specialist are required.
Treatment
The primary goal of pemphigus treatment is to stop new blisters and erosions from forming while also promoting the healing of already damaged skin. Glucocorticosteroids are the primary medications used in the treatment of pemphigus. Regardless of the baby’s age, a course of systemic glucocorticosteroid medication is prescribed as soon as the diagnosis is verified in the lab.
Doses of the medications are increased. This aids in lessening the force with which new blisters form and initiates the process of repairing already-existing erosions. This typically takes two weeks, following which the child receives the same medication in small doses for an extended period of time as part of maintenance hormonal therapy.
"Prednisolone" has been shown to be the most successful medication. It is given at higher dosages for vulgar pemphigus than for foliaceous. After that, the dosage of the medication is gradually decreased until a maintenance level is reached. Unfortunately, this type of treatment will be ongoing for the great majority of patients, requiring daily injections of "Prednisolone."
In addition to hormones, the doctor gives the child prescriptions for calcium supplements and vitamin D. Immunosuppressive medications, which artificially suppress the immune system, are prescribed from the outset of treatment to maximize its efficacy. In pediatric practice, these medications include:
- "Azathioprine";
- "Myelosan";
- "Cyclophosphamide";
- "Cyclophosphamide".
Early on in the course of treatment, the child might be given instructions on how to "cleanse" the blood—if only momentarily—of a lot of aggressive antibodies. These treatments consist of plasmapheresis and hemodialysis. The child’s skin (affected areas) will be treated with ointments containing corticosteroids and antiseptics to lessen the risk of infection.
It should be made very clear to parents that pemphigus treatment is probably lifelong. In extremely rare circumstances, with brief intervals between relapses.
Child care
Children who are diagnosed with "pemphigus" need daily care that is more attentive and requires special attention. How well the parents manage their infant’s life will determine how long the child can live. It is crucial to make sure the infant continues to take all of the prescription drugs at home after being released from the hospital, where the first phase of treatment is being administered.
The family budget will be severely damaged by using paid medical services at home on a daily basis, so mom or dad will need to learn how to administer injections.
The child’s eczema and skin blisters must be treated daily. Use of aniline dyes (fucorcid, brilliant green) is advised for this, as they are particularly effective against staphylococcus and other microbes. When skin erosions and crusts form, it is advised to apply a corticosteroid ointment. Typically, "Celestoderm" is prescribed along with "Garamycin" or "Gioxizon." Another common usage is a dermatol ointment with a 5% active ingredient concentration.
When pus, inflammation, or swelling indicate an infection, it is critical to apply antibiotic-containing ointments ("Baneocin," "Levomekol"). If the injured areas are large, it is best to cover them with a sterile bandage to prevent further damage. It’s recommended to dress at least twice a day. In cases where the child is highly active and mobile, it makes sense to apply dressings even for minor lesions.
Refusing to give analgesics (Ibuprofen or Paracetamol) to a child who complains of pain is preferable to giving them anti-inflammatory drugs (Analgin). It is essential to see a physician who can prescribe analgesic medications that are safe for use in children if pain relief is not achieved. Make sure the child washes his mouth several times a day with antiseptic solutions in case new pemphigus elements and erosions appear in the mouth.
The baby will benefit from baths to which parents add antiseptic solutions, for example, chlorhexidine. It is advisable for the child to take vitamin and mineral complexes that contain folic acid, vitamin E, calcium and magnesium. You will have to make changes to your child"s diet. The child should eat small portions and fractionally (up to 6 times a day). This is especially important if pemphigus has affected the mucous membranes in the mouth, in the esophagus. If you do not give your child pureed soups and porridges, which are relatively easy to eat, then he may refuse food altogether due to pain, and then you will have to feed him through a tube. The diet of a child with pemphigus should be based on the refusal of salty foods, you should completely exclude salt from the recipe for all dishes, however, the baby will need a large amount of protein food.
At the clinic where they live, the child must be registered with a dermatologist. This specialist will need to see you once every six months, and on an as-needed basis if there is a relapse and the condition gets worse. Both therapy and dispensary registration are typically lifelong.
For a child taking medication to suppress immune function, many vaccinations are not recommended. However, parents have the option to vaccinate their child against pneumococcal and influenza. A baby who has pemphigus should use caution when swimming and tanning. This is only possible during a period of remission, during which the skin is free of new blisters and eczema. Children with severe forms of the disease are typically homeschooled, and in certain instances, they are classified as disabled.
A child with pemphigus can successfully engage in any type of mental work, as it has no effect on the child’s mental or intellectual development. Sort by Category Limiting the child’s exposure to allergens and toxic substances, as well as household chemicals, is recommended to avoid situations where the infant could be burnt or injured. Moreover, acute respiratory viral infections ought to be prevented.
In times when there is a widespread surge in the occurrence of acute respiratory viral infections, it is advisable for a child suffering from pemphigus to avoid crowded areas such as stores and clinics.
Forecasts
Pemphigus is thought to have a conditionally unfavorable prognosis. Unfortunately, the likelihood of a fatal outcome does not go away even if treatment is started on time and medications are prescribed appropriately and competently. In any form, the disease is always regarded as chronic. Prior to the use of hormonal agents, specifically glucocorticosteroids, in the management of the illness, the risk of death in pemphigus was close to 65%. Experts now estimate that the chance of these unfavorable events is between 6.5 and 7%.
About 9% of patients with this diagnosis experience persistent remission, or the absence of all disease symptoms, in the absence of ongoing maintenance therapy. In the 91% of patients who do not receive treatment, there is an exacerbation that results in a substantial worsening of the condition and raises the possibility of a fatal outcome.
| Type of Pemphigus | Symptoms |
| Pemphigus Vulgaris | Blisters appear in the mouth first, then spread to the skin. |
| Pemphigus Foliaceus | Blisters form on the skin, usually starting on the scalp and face, no mouth involvement. |
| Neonatal Pemphigus | Blisters appear on newborns, often caused by maternal antibodies. |
| Pemphigus Herpetiformis | Itchy, grouped blisters similar to herpes, but not caused by the herpes virus. |
Parenting with a child who has pemphigus can be difficult, especially if the child is a newborn or infant. Effective disease management and the avoidance of complications depend on early detection.
Parents can help guarantee their child receives the proper treatment by identifying the symptoms, such as blisters on the skin and mucous membranes, and getting medical help as soon as possible. This will ease pain and encourage recovery.
Most cases can be successfully handled with the right care, enabling kids to heal and prosper. It’s critical to closely adhere to medical advice and keep an eye on your child’s condition as they recover.
