Bullous pemphigoid is a relatively common skin disease that resembles pemphigus in appearance. The disease proceeds in a chronic form and in the absence of timely diagnosis and treatment can lead to unpleasant consequences. So what causes the development of such a disease? What symptoms does it manifest? What treatments can modern medicine offer? The answers to these questions are of interest to many readers.
What is a disease?
Bullous pemphigoid in modern medicine is known by many names - this is Lever's disease, and senile pemphigus, and senile dermatitis herpetiformis. This is a chronic autoimmune disease that is accompanied by the appearance of a large blistering rash on the skin (external symptoms sometimes resemble true pemphigus).
It is worth noting that the overwhelming majority of patients with this diagnosis are people aged 65 and older. Naturally, exceptions are known to medicine, since the disease is sometimes found in children and middle-aged patients. diseasethis is characterized by a benign course, but can sometimes lead to complications. In the clinical picture, periods of relative well-being alternate with exacerbations. Of course, for many people, the question of what constitutes bullous pemphigoid is interesting. Symptoms and treatment of the disease, the causes of its occurrence - this information should be read more carefully.
Some related diseases
It is worth noting that bullous pemphigoid is included in the group of so-called blistering dermatoses. These ailments differ from true pemphigus, as they are not accompanied by acantholysis. The group of skin lesions includes several other ailments, the clinical picture of which is quite similar:
- Benign non-acantholytic pemphigus, in which the ailment affects exclusively the mucous membrane of the mouth, without causing a rash in other areas. The disease is also characterized by a benign course. By the way, it was first described in 1959.
- Scarring pemphigoid is a rather dangerous disease that affects the mucous membrane of the eyes and conjunctiva, causing its atrophy. Rashes on the body are possible, but are relatively rare. The main risk group is women aged 50, although sometimes the disease is also recorded among male patients.
Causes and pathogenesis of bullous pemphigoid
Unfortunately, the mechanism of this disease is not yet fully understood. Nevertheless, scientists managed to find out that the disease has an autoimmune character. For one reason or another, failures occurimmune system, resulting in produced antibodies attack not only foreign, but also the body's own cells.
Evidence of this theory is available. During studies in the patient's blood serum, as well as in the fluid taken from the blisters, specific antibodies were found that damage the basement membrane of the tissues of the skin and mucous membranes. It was also possible to establish that the more actively the disease develops, the higher the titer of these antibodies.
It is believed that autoimmune diseases are genetically determined. However, a factor capable of activating the disease is required. It could be:
- vaccination against certain diseases;
- damage or severe skin irritation;
- exposure to ultraviolet radiation (prolonged sunbathing, tanning bed abuse, etc.);
- thermal skin burns;
- frequent use of certain medications, such as Furosemide, Captopril, Phenacetin, Amoxicillin and some others;
- sometimes the disease is activated after a patient undergoes radiation therapy;
- kidney transplant rejection, repeated organ transplants.
Bullous pemphigoid: photos and symptoms
Of course, first of all, it is important to get acquainted with the symptoms, because the sooner the patient pays attention to the presence of disorders and consults a doctor, the easier the treatment process will be. The formation of tense blistering rashes on the skin is the main symptom that accompanies bullouspemphigoid (the photo shows what the rash looks like). Most often, the skin of the extremities and trunk is affected. Rashes can occur in the area of large natural folds, on the skin of the face and head, but this occurs less frequently.
The main elements of the rashes are vesicles and blisters with tight tires. Inside they contain a liquid, usually transparent, but sometimes you can see blood impurities. Often, the skin around the blisters turns red.
The "life" of formations is several days. After that, they spontaneously open. At the site of the rash, areas of erosion and small sores are formed. Crusts are practically not formed on the surface, since the erosive areas quickly epithelialize.
The first stages of the development of the disease in 20% of patients begin with the appearance of bubbles on the mucous membrane of the oral cavity, and only then the rash passes to the skin. Blisters on the mucous membrane of the nose, pharynx, genitals, eyes appear extremely rarely.
Patients complain of itching, and after opening the blisters and of some soreness. An increase in temperature is possible, although this is rare. Elderly patients, whose body is depleted by frequent relapses, also experience decreased appetite, weight loss, and progressive weakness.
Histogenesis, histopathology and pathomorphology
The pathology of bullous pemphigoid is quite interesting. First, numerous vacuoles form between the cytoplasmic processes of the basal cells. Gradually, these formations merge with each other, forming largerstructures. Along with this, there is a sharp swelling of the tissues of the dermis.
The bladder lid is an epidermal tissue. Its cells are stretched, but the bridges between them are not damaged. As the disease progresses, the cells of the epidermis gradually die off. At the same time, new epidermal tissues move up from the edges of the bubble, capture its bottom - thus, the vesicle moves inside the epidermis, and sometimes into the substratum.
Inside the bladder there is a fluid that contains lymphocytes mixed with neutrophils. There are fibrin threads, protein molecules and some other compounds.
If we consider the histogenesis of bullous pemphigoid, then first it is worth remembering that the disease is autoimmune. When examining tissues using an electron microscope, it can be seen that the so-called BPAg1 antigens, which are released during the immune response, are located in the basal layer, namely at the attachment sites of keratinocyte hemidesmosomes. Another antigen, BPAg2, is also located in the hemidesmosome region. It is believed to be formed by type XII collagen.
Also in the process of research, it was found that macrophages and eosinophils in this disease first accumulate at the basement membrane, after which they migrate through it and begin to accumulate inside the bladder and between the basal cells. There is also significant mast cell degranulation.
Histologically, in the disease, there is a detachment of the epidermis from the dermis, between which a subepidermal bubble is formed. Vessels in the skintissues are also expanded, swelling of their inner layers (endothelium) is observed.
Modern diagnostic methods
As a rule, there are no difficulties in diagnosing such a disease as bullous pemphigoid: the symptoms here are very characteristic, and therefore the doctor may suspect the disease already during a standard examination. Tension blisters form on the patient's skin, and the process of erosion epithelialization proceeds quickly.
Epidermis peel test negative. Additionally, the internal contents of the blisters are taken with further histological examination. During laboratory tests, vacuoles, histiocytic elements, eosinophils and lymphocytes can be detected in the fluid.
On the other hand, the differential diagnosis is sometimes difficult, as the clinical picture slightly resembles other skin diseases, including erythema multiforme exudative, pemphigus vera, and Dühring's herpetiformis.
Which treatment is considered effective?
What to do if you have bullous pemphigoid? Treatment in this case requires complex. Moreover, the selection of he alth-improving measures and medications depends on many factors, including the severity of the disease, the age and general he alth of the patient, and the presence of concomitant pathologies. In any case, the treatment regimen can only be drawn up by the attending physician.
The basis of therapy is steroid anti-inflammatory drugs containing glucocorticosteroids. Most often, Prednisolone is used for this purpose. The medicine is being injectedintravenously, and the dose is gradually reduced as symptoms disappear.
Cytostatics and immunosuppressants also give a good effect, which help to normalize the functioning of the immune system. Quite often, patients are prescribed drugs such as Cyclosporine A, Cyclophosphamide, Azathioprine.
Naturally, the treatment of rashes, erosions and sores on the skin is also an important point. You need to keep your skin clean. Patients are prescribed solutions with aniline dyes (for example, Furkotsin), which act as antiseptics, drying the skin. In more severe cases, steroid ointments are also required.
Treatment with folk remedies
Bullous pemphigoid, or Lever's disease, is a pathology that requires competent, qualified treatment. The use of various home-made medicines is possible, but only with the permission of a specialist. Before using any remedy, be sure to consult your doctor. In folk medicine, many different medicines are used.
- It is believed that Eleutherococcus tincture will positively affect the patient's he alth. Take it twice a day, 30 drops each.
- For external treatment of rashes, aloe leaf juice is used, which helps relieve itching and soreness, prevents the development of the inflammatory process, and accelerates regeneration processes. Moisten the bandage with juice, then apply it to the damaged area of \u200b\u200bthe skin and secure with a bandage. For maximum effect, you can covercompress with plastic wrap.
- For the same purpose, fresh juice or decoction of nettle leaves can be used. The compress is done as described above.
- Bullous pemphigoid, more precisely, its symptoms can be alleviated with the help of a special herbal decoction. For its preparation, take an equal amount (50 g each) of eucalyptus leaves, serpentine rhizomes, Japanese Sophora fruits, birch buds, yarrow grass, shepherd's purse and nettle. Pour two tablespoons of the prepared mixture of herbs in the evening with a glass of boiling water and leave overnight. In the morning, the infusion should be filtered and divided into three portions - they are taken throughout the day.
It should be understood that herbal medicines for each patient can act differently. Even if the remedy has a positive effect, in no case should you refuse drug therapy.
Prognosis for patients
Pemphigoid is a benign skin disease, and therefore, in most cases, it does not proceed too hard. Moreover, in almost any hospital in a large city, the disease is successfully treated under such a complex name - bullous pemphigoid. In Orenburg, Moscow and any other city you will definitely find a good specialist. Only the cost of therapy will depend on the place of residence, since the prices for certain drugs in different pharmacies vary.
With the right treatment, it is possible to achieve a stable remission. From time to time, some patients have relapses, which, of course, is unpleasant, but alsonot fatal. On the other hand, in the absence of therapy, the sites of rash formation can become a gateway for infection, which, accordingly, ends in a more massive inflammatory process, suppuration of wounds, and the penetration of pathogenic bacteria into the deeper layers of the skin.
Are there preventive measures?
Unfortunately, there is no specific remedy for the prevention of such a disease as Lever's bullous pemphigoid. Naturally, it is extremely important to seek help in time, and since the disease is chronic, even in periods of relative well-being, one must carefully monitor the state of he alth.
Do not forget that the disease in medicine is regarded as a possible marker of oncology. Therefore, in the presence of an ailment, the patient must necessarily undergo a comprehensive examination in order to confirm or exclude an oncological diagnosis. Remember that any disease is much easier to deal with if you start therapy at an early stage.
