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 an ailment? What symptoms does it manifest? What remedies can modern medicine offer? The answers to these questions are interesting to many readers.
What is a disease?
Bullous pemphigoid in modern medicine is known under many names - this is Lever’s disease, and senile pemphigus, and senile herpetiform dermatitis. This is a chronic autoimmune disease, which 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 vast majority of patients with this diagnosis are people aged 65 years and older. Naturally, exceptions are also known to medicine, since the disease is sometimes found in children and middle-aged patients. This disease is characterized by a benign course, but sometimes it can 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 a pemphigoid bullous is interesting. Symptoms and treatment of the disease, the causes of its appearance - this information should be read more carefully.
Some similar diseases
It is worth noting that bullous pemphigoid is part of the group of so-called cystic dermatoses. These ailments differ from true pemphigus, as they are not accompanied by acantholysis. The group of skin lesions includes several more ailments, the clinical picture of which is quite similar:
- Benign non-acantholytic pemphigus, in which the disease only affects 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 observed relatively rarely. The main risk group is women of 50 years of age, 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 were able to find out that the disease has an autoimmune nature. For one reason or another, malfunctions of the immune system occur, as a result of which the produced antibodies attack not only foreign, but also the body’s own cells.
Evidence for this theory is available. During studies in the patient's blood serum, as well as in fluid taken from the blisters, specific antibodies were found that damage the basement membrane of skin tissues and mucous membranes. It was also possible to establish that the more actively the disease develops, the higher the titer of these antibodies.
Autoimmune diseases are believed to be genetically determined. Nevertheless, 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, abuse of a solarium, etc.);
- thermal skin burns;
- frequent use of certain medications, for example, Furosemide, Captopril, Phenacetin, Amoxicillin and some others;
- sometimes the disease is activated after the patient undergoes a course of radiation therapy;
- renal transplant rejection, repeated organ transplants.
Bullous pemphigoid: photos and symptoms
Of course, in the first place 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 intense blistering rashes on the skin is the main symptom that accompanies a bullous pemphigoid (photo shows what the rash looks like). Most often, the skin of the limbs and trunk is affected. Rashes can occur in the area of large natural folds, on the skin of the face and head, but this happens less frequently.
The main elements of the rashes are vesicles and blisters with strained tires. Inside, they contain liquid, usually transparent, but sometimes you can notice blood impurities. Often the skin around the vesicles turns red.
The term of “life” of formations is several days. After that, they spontaneously open. At the site of rashes, erosion patches and small sores are formed. Crusts on the surface practically do not form, since erosive areas are rapidly epithelized.
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 some pain. A fever is possible, although this is rare. In elderly patients, whose body is exhausted by frequent relapses, there is also a decrease in appetite, weight loss, progressive weakness.
Histogenesis, histopathology and pathomorphology
The pathomorphology of the bullous pemphigoid is quite interesting. First, numerous vacuoles are formed between the cytoplasmic processes of the basal cells. Gradually, these formations merge with each other, forming larger structures. Along with this, there is a sharp swelling of the dermis tissue.
The bubble of the bladder is epidermal tissue. Her cells are stretched, but the bridges between them are not damaged. As the disease develops, the epidermal cells gradually die off. At the same time, new epidermal tissues advancing from the edges of the bladder, capturing its bottom - thus, the vesicle moves inside the epidermis, and sometimes into the subuterine layer.
Inside the bladder there is a fluid that contains lymphocytes mixed with neutrophils. Fibrin filaments, protein molecules and some other compounds are present.
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, one can notice that the so-called BPAg1 antigens that are released during the immune response are located in the basal layer, namely, in the places where keratinocytes are attached by semi-desmos. Another antigen - BPAg2 - is also located in the semi-desmos region. It is believed that it is 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. Significant mast cell degranulation is also observed.
Histologically, with the disease, exfoliation of the epidermis from the dermis is observed, between which a subepidermal bladder is formed. The vessels in the skin tissues are also dilated, swelling of their inner layers (endothelium) is observed.
Modern diagnostic methods
As a rule, there are no difficulties with the diagnosis of a disease such as bullous pemphigoid: the symptoms are very characteristic, and therefore the doctor may suspect an ailment already during a standard examination. Tense blisters form on the patient's skin, and the process of epithelialization of erosion proceeds quickly.
The test for exfoliation of the epidermis is negative. Additionally, a sampling of the internal contents of the blisters is carried out with further histological examination. During laboratory tests, vacuoles, histiocytic elements, eosinophils and lymphocytes can be found in the fluid.
On the other hand, the differential diagnosis is sometimes fraught with difficulties, since the clinical picture slightly resembles other skin diseases, including erythema multiforme exudative, true pemphigus and Dühring's herpetiform dermatitis.
What treatment is considered effective?
What to do if you have found a bullous pemphigoid? Treatment in this case requires a comprehensive. Moreover, the selection of recreational activities and medications depends on many factors, including the severity of the disease, the age and general health of the patient, the presence of concomitant pathologies. In any case, the treatment regimen can only be compiled by the attending physician.
The basis of therapy is steroidal anti-inflammatory drugs containing glucocorticosteroids. Most often, Prednisone is used for this purpose. The medicine is administered intravenously, and the dose gradually decreases as the symptoms disappear.
Cytostatics and immunosuppressants, which help normalize the functioning of the immune system, also give a good effect. Quite often, patients are prescribed drugs such as Cyclosporin A, Cyclophosphamide, Azathioprine.
Naturally, an important point is the treatment of rashes, erosions and sores on the skin. Keep your skin clean. Patients are prescribed solutions with aniline dyes (for example, "Furcocin"), which act as antiseptics, drying the skin. In more severe cases, ointments with steroids 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 product, be sure to consult your doctor. In folk medicine, many different medicines are used.
- It is believed that tincture of Eleutherococcus will positively affect the patient's state of health. You need to take it twice a day, 30 drops.
- For external treatment of the rash, juice from aloe leaves is used, which helps to relieve itching and soreness, prevents the development of the inflammatory process, accelerates the regeneration processes. You need to moisten the bandage with juice, then apply it to the damaged area of the skin and fix it with a bandage. To achieve maximum effect, you can cover the compress with a plastic film.
- For the same purpose, fresh juice or a decoction of nettle leaves can be used. Compress is done according to the above scheme.
- Bullous pemphigoid, more precisely, its symptoms can be mitigated with the help of a special herbal decoction. To prepare it, take an equal amount (50 g) of eucalyptus leaves, serpentine rhizomes, Japanese Sophora fruits, birch buds, yarrow grass, shepherd’s bag and nettle. Two tablespoons of the prepared mixture of herbs in the evening pour a glass of boiling water and leave overnight. In the morning, the infusion should be filtered and divided into three servings - they are taken throughout the day.
It should be understood that herbal remedies for each patient can act differently. Even if the drug has a positive effect, in no case should you give up drug therapy.
Prognosis for patients
Pemphigoid is a benign skin disease, and therefore it proceeds in most cases not too hard. Moreover, in almost any hospital in a large city, a successful treatment of an ailment under such a complex name - bullous pemphigoid is carried out. In Orenburg, Moscow and any other city you are sure to find a good specialist. Only the cost of therapy will depend on the place of residence, since the prices for certain drugs vary in different drugstores.
With the right treatment, stable remission can be achieved. From time to time, some patients have relapses, which, of course, is unpleasant, but not fatal. On the other hand, in the absence of therapy, the site of the formation of rashes can become a gateway to infection, which, accordingly, ends with a more massive inflammatory process, suppuration of wounds, and the penetration of pathogenic bacteria into deeper layers of the skin.
Are there any preventative measures?
Unfortunately, there is no specific tool for the prevention of a disease such 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, you need to carefully monitor your health.
Do not forget that the disease in medicine is regarded as a possible marker of oncology. And therefore, if there is an ailment, the patient must undergo a comprehensive examination to confirm or exclude the oncological diagnosis. Remember that it is much easier to cope with any disease if you start therapy at an early stage.