Sometimes people develop blistering rashes on their skin that vaguely resemble burns. This is pemphigoid, a benign disease of unknown autoimmune etiology.

General description of the disease

There is no clear position among scientists regarding the causes of the disease, so there are numerous hypotheses about the supposed development factors. But all dermatologists agreed in one opinion: the initial mechanism for the occurrence of pemphigoid is an autoimmune process. This has been confirmed by various scientific studies: histological, biochemical, immunological.

There is some justification for the allergic nature of the disease, since antibodies of different classes are detected to the basement membrane of the skin layers.

At an early stage of development, the disease manifests itself by the formation of microbubbles (microvacuoles) on the patient’s skin. In the future, the formation of large bubbles may occur, which is caused by their fusion. The epidermis separates from the dermis. Subsequently, the top layer becomes necrotic and almost completely destroyed.

The blisters usually regress spontaneously. However, in some cases (this depends on the type of pemphigoid itself), the formation of fibrosis of the upper skin tissues is possible. In this case, the elasticity of the skin is lost. When the bladder is artificially opened, erosion may form.

The localization of bubbles is different. These can be either limited areas of the body or wide areas of distribution of the manifestations of the disease. The location of the lesion depends on the type of disease.

Classification of the disease

Pemphigoid is not a single disease, but a group of diseases, the so-called vesicular dermatoses, which, unlike true pemphigus, are not accompanied by degenerative changes in the spinous layer of the epidermis in the form of loss of connections between epidermal cells (acantholysis). Such dermatitis is often called non-acantholytic pemphigus.

There are three main types of this disease:

1. Bullous pemphigoid, often called Lever's pemphigoid, parapemphigus, bullous dermatitis herpetiformis, benign pemphigus vulgaris, classic non-acantholytic pemphigus.
2. Cicatricial pemphigoid, also known as: mucosynechial bullous dermatitis, ocular pemphigus, conjunctival pemphigus.
3. Benign non-acantholytic pemphigus of the oral and nasal mucosa or benign pemphigoid of the mucous membranes.

There is the so-called pyococcal or staphylococcal pemphigoid, which is a completely different type of disease. This is a skin infection, usually caused by Staphylococcus aureus, that develops in newborn babies in the first days of life. This disease poses a real danger to the health and life of the child.

Bullous pemphigoid (photo)

Bullous pemphigoid is a benign dermatological disease that has a chronic course.

This is the most common and at the same time severe form of this type of disease, developing mainly in people over 60 years of age. Men are more likely to suffer from this type of disease than women.

Varieties

There are two main types of this pemphigoid:

1. Generalized (generalized), occurring most often. In this case, bullae can form in any place, but the usual place of initial localization is the area around the joints. The mucous membrane is rarely involved in the lesion process. The healing process occurs without the appearance of scars.
2. Vesicular, less common subspecies. It is characterized by the appearance of small groups of tense blisters, which are localized on erythematous (reddened, covered with a fused rash) areas of the skin. In this case, the mucous membrane is involved in the process. The pathology results in blisters, scars, and erosions.

There are rare forms of bullous pemphigoid:

• acral or juvenile childhood variety, usually occurring after vaccination;
• nodular, with the formation of itchy nodular-type lesions;
• eczematous, resembling eczema in appearance;
• vegetative, with the formation of interfold plaques;
• erythrodermatic, in which the filling of the bullae is dark in color due to the presence of a large number of red blood cells in it;
• dyshydrosiform, with a predominant localization of blisters on the soles of the feet and palms, reminiscent of atopic eczema.

Clinical picture of the disease

In the early stages of the disease, the clinical picture is extremely variable. Bulls appear only at a later stage. And visible symptoms manifest themselves during the period of acute and subacute onset of the disease. These are signs of bullous pemphigoid, such as:

• itching, which manifests itself long before visible skin lesions (sometimes several months);
• urticaria () or erythermatous (red) rash preceding the appearance of blisters;
• blisters (bullas) can develop locally, within one lesion, or spread throughout the body;
• seizure of mucous membranes (in ¼ of patients), with the possible manifestation of dysphagia - impaired swallowing.

Subsequently, bullous rashes appear, the size of which ranges from 0.3 to 5 cm in diameter. In some cases, itching in the areas of the rash may give way to pain.

All bubbles are located symmetrically relative to the midline of the body. The skin at the affected sites is usually inflamed and swollen. Initially, the filling of the bullae is transparent, but over time, purulent or bloody contents may appear. After spontaneous opening of the blisters, the area heals quickly, sometimes without the formation of scars and crusts.

The general condition of a person has no disturbances, that is, there is no depressed state: consciousness, muscle tone and the functioning of internal organs are normal. As a rule, body temperature does too. There is no vomiting, diarrhea, or nausea.

However, since this disease is a disease of older people, patients in this group may experience decreased appetite, muscle weakness, and weight loss.

The disease has a chronic course: a period of absence of symptoms (remission) alternates with a time of resumption of symptoms (exacerbation), and with each new round the pathology spreads more and more widely. Relapses are often associated with exposure to UV rays on the patient's body.

Causes

It is believed that the following factors can stimulate the occurrence of the disease:

• uncontrolled use (self-medication) of medications, such as:
  • Furosemide;
  • Penicillins;
  • Phenacetin;
  • Ciprofloxacin;
  • Captopril;
  • Salazopyridazine;
  • Fluorouracil;
  • Potassium iodide.
• ultraviolet irradiation;
• tumor processes of various localizations.

Related ailments

Often the disease in elderly people is combined with the development of oncological tumors and neuralgic diseases:

• Parkinson's disease;
• dementia;
• paralysis;
• bipolar disorders;
• Alzheimer's disease.

Diagnosis of bullous pemphigoid

To diagnose the disease, a comprehensive examination of patients is used, including not only general tests, but also complex studies of biomaterials.

1. A biopsy is performed with a complete sample taken, including the base of the vesicle.
2. Immunohistological study of the epithelium.
3. Isolation of blood serum with detection of autoantibodies.
4. Immunofluorescence reaction with determination of accumulation of immunoglobulins of different types.

Additionally, the following laboratory techniques are used:

• Nikolsky's symptom (exfoliation test with minor mechanical impact is negative, in contrast to true pemphigus);
• light and electron microscopy, revealing:
  • infiltration of the dermis (pronounced accumulation of fluid, accompanied by inflammatory processes);
  • epidermal cleft (loss of connections between epidermal cells);
  • mononuclear infiltration (accumulation of monocytes and lymphocytes at the site of inflammation);
  • the presence of a protein that occurs under the influence of blood clotting in the cavity of the bladder;
• immunoelectron microscopy (with gold labeling);
• immunochemical studies.

It is necessary to differentiate pemphigoid from a number of other dermatoses:

The basis for such differentiation is a comparison of the results of one of the biochemical studies in the absence of the following criteria:

• skin atrophy;
• lesions of the head and neck.

Treatment of the disease

Treatment of bullous pemphigoid is prescribed comprehensively and only on an individual basis.

The basis of the therapeutic effect is the use of glucocorticosteroids intravenously/intramuscularly and externally. Usually this is Prednisolone.

In parallel, immunosuppressants are prescribed, which are selected in accordance with the general condition and age of the patient. Typically used in treatment:

• Methotrexate;
• Chlorambucil;
• Cyclosporine;
• Azathioprine;
• Cyclophosphamide.

If the use of corticosteroids is contraindicated, combinations of Nicotinamide and Minocycline may be used. Treatment with Dapsone is possible.

During therapeutic exposure, it is necessary to minimize the risk of complications: gastrointestinal diseases, deterioration of heart function. In case of possible secondary infection, the use of antibacterial drugs is required.

If left untreated, sepsis may develop against the background of a possible immune disorder, which can be fatal for a depleted body.

Pemphigoid cicatricial

Symptoms

Cicatricial pemphigoid, more commonly known as pemphigus oculi, a disease of the mucous membranes of the conjunctiva. It happens that during the pathological process the membranes of the mouth and genital organs are affected, and sometimes the skin is involved. If there is no treatment, after a few years the disease can affect the mucous membranes of the larynx, esophagus, and urinary tract.

The size of the resulting bubbles ranges from 1 mm to 50 mm in diameter. The lesions are hyperemic (reddened due to a rush of blood), located in groups and often recur in the same place, which leads to roughening of the skin and the appearance of scars.

The filling of the blisters is serous, and the erosions that appear after opening the bullae have a fibrinous coating.

Damage can begin in different parts of the body:

1. The pathology of the conjunctiva begins with ordinary inflammation, accompanied by the release of mucous purulent exudate. Then, against the background of redness of the eyes, blisters appear, which quickly open. Erosion occurs, bleeding and painful. Over time, erosions become covered with plaque. Scarring changes occur, as a result of which the conjunctiva and the eyeball grow together. If left untreated, ulceration and blindness may occur.
2. Damage to the mucous membranes of the oral cavity can lead to adhesions between the lining of the cheeks and the alveolar processes, in the corners of the mouth. There is a possibility of destruction of the tonsils.
3. With the development of pathology in the nasopharynx, the onset of atrophy and the formation of strictures of the larynx and esophagus are possible. Lack of treatment leads to fusion of the nasal turbinates with the nasal septum.
4. The occurrence of foci of the disease in the larynx is accompanied by periodic coughing with the separation of bloody films (tire blisters).
5. On the skin, localization of lesions occurs on the face, limbs, and inguinal folds. Bubbles are single and persistent. Erosion heals slowly. If the affected area is the scalp, the result is baldness.

Cicatricial pemphigoid most often occurs in women over 50 years of age and children. Usually the disease continues until the end of life, but with proper treatment there is a possibility of loss of activity and further development of the disease itself.

It must be remembered that if a lesion is detected in one eye, then there is a high probability of a similar manifestation of the disease in the other.

Diagnosis of the disease

Diagnosis of the disease at its initial manifestation is an extremely difficult process when the localization of lesions is outside the mucous membranes. Only during the period of scarring is it possible to differentiate the disease from bullous pemphigoid and pemphigus. Electron microscopy is usually used.

The diagnosis is established on the basis of the obvious clinical picture in accordance with the confirmed results of histological examination. The detection of blisters on the mucous membranes in combination with conjunctival lesions is an additional confirmatory factor in the presence of cicatricial pemphigoid.

Examinations and consultations with medical specialists will help determine the etiology of the disease:

• dermatologist;
• otolaryngologist;
• dentist;
• infectious disease specialist

Therapeutic measures

Treatment of the disease depends on its location.

For skin tissue lesions, glucocorticoids (usually Prednisolone) and immunosuppressants (Azathioprine and Cyclophosphamide) are used. In such cases, the treatment of cicatricial pemphigoid has a common principle with the therapeutic effect in the bullous form of the disease.

Lesions of the conjunctiva and other mucous membranes require a different approach.

1. Eye disease is treated with:
   1. eye drops containing dexamethasone;
   2. antibacterial and antiseptic drugs: Okomistin, Levomycetin drops, Miramistin solution 0.01%;
   3. healing agent - Korneregel.
2. When the disease manifests itself in the mucous membranes of the mouth and nose, the following is indicated:
   1. use of symptomatic therapy: Subcutin, Dinexan, Herviros, Kavosan;
   2. local glucocorticoids: Volon A, Dontisolone, crystalline suspension of Triamcinolone;
   3. For disinfection, rinsing with antiseptics is prescribed: Miramistin, Chlorhexidine, an aqueous solution of Furacilin.
   4. In case of severe tissue damage, skin transplantation is possible.

In the absence of regeneration of lesions located outside the mucous membranes, doctors often prescribe combination medications consisting of hormones and antibiotics: Pimafucort, Aurobin, Imakort. In addition to the main treatment, when the lesions do not heal and there is no exact cause of the disease, Bepanten cream, Methyluracil and Levomekol gel are prescribed.

Alas, there are no established preventive measures to prevent the occurrence of bullous and cicatricial pemphigoid. If you follow all the recommendations and prescriptions of the attending physician, the prognosis for future life (not for health!) is always favorable. In this case, of course, the age of the patient and his general condition should be taken into account.

Benign non-acantholytic pemphigus of the oral mucosa

An independent form is considered benign non-acantholytic pemphigus of the oral mucosa only. Bubbles appear only on the surface of the mouth, are minimal in size, and are not accompanied by pain. After damage to the bullae, erosion occurs, which heals without leaving marks within 7–15 days.

The main characteristic of this type of disease is the chronic course of the disease followed by spontaneous disappearance of all symptoms. Only in extreme cases is it possible for a secondary infection to occur, which is treated in accordance with the disease that has arisen.

Gestational pemphigoid

There is a certain type of disease that occurs during pregnancy. This disease is called gestational pemphigoid or gestational herpes (although this disease has nothing to do with the herpes virus).

Water bubbles usually appear in the 3rd trimester of pregnancy. However, rashes are possible at any stage, and even a month and a half after childbirth.

This is a very rare dermatosis caused by autoimmune processes in the body of a pregnant woman who has leukocyte antigens of a certain type (DR3 and DR4). The probability of the disease occurring is 1 case in 50 thousand women. Having appeared during the first pregnancy, the disease usually manifests itself in subsequent pregnancies.

Symptoms

In most cases, the localization of the rash is formed in the area of ​​the umbilical part of the abdomen, but sometimes the rash spreads throughout the woman’s body. The mucous membranes are usually not infected and without damage.

The manifestation of gestational pemphigoid is characterized by:

• intense itching;
• papules;
• irregularly shaped plaques;
• blisters and vesicles with serous filling.

The obvious manifestation of the disease lasts for several weeks, worsening during childbirth. There are cases of prolonged remission, as well as relapses that manifest themselves during menstruation.

In the absence of proper treatment, the disease may affect the health of the unborn child. The baby is born prematurely, with possible vesicular rashes that disappear during the first month of life.

Treatment

Therefore, it is very important to correctly diagnose it at an early stage of the onset of symptoms of the disease and begin outpatient treatment. General principles of therapy:

• preventing new rashes;
• relieving itching;
• preventing secondary infection.

For this use:

• Triamcinolone acetonide cream;
• Prednisolone cream or ointment (for extensive rash);
• antihistamines;
• infusion therapy (intravenous or subcutaneous administration of medications to normalize the condition);
• plasmapheresis (a special blood filtration procedure).

Nutritional Features

When treating any type of disease, certain nutritional conditions and regimens are required.

During the recovery period, it is necessary to avoid excessive physical activity, preferring a calm, measured lifestyle. Doctors often advise bed rest.

Following a special diet is part of the treatment. The following dietary recommendations should be followed:

• exclude fatty fish and meat from the diet;
• give up sweets and baked goods;
• increase the amount of vegetables, fruits, herbs and natural juices consumed;
• reduce consumption of dairy products;
• minimize the consumption of canned foods and sausages in your diet;
• exclude sauces and mayonnaise from the diet;
• Avoid potentially allergenic foods (for example, oranges, raisins, grapes).

The patient’s daily menu should always include:

• porridge;
• whole grains;
• lean broths;
• fresh salads;
• seafood and sea fish;
• lean meat.

It is recommended to consume steamed, baked and boiled dishes. A prerequisite on the path to recovery is an increased drinking regime, where preference should be given to natural hypoallergenic juices, compotes, teas, and mineral waters. The volume of liquid consumed should be at least two liters.

Use of traditional medicine

Any type of pemphigoid is a dangerous pathology that requires adequate treatment. Moreover, in addition to it, with the permission of the attending physician, you can use folk remedies. Usually these are decoctions and juices of plants used as disinfectants and regenerators.

1. Compress with aloe juice. The clean, fleshy leaf of the plant is peeled, crushed and the juice is squeezed out. A gauze napkin is thoroughly soaked in the resulting juice and applied to the affected areas for 20–30 minutes.
2. Compresses with nettle juice. Fresh leaves of the plant are crushed and passed through a juicer. Soak a napkin with the resulting liquid mass and apply it to the lesions for 10–20 minutes.
3. Herbal decoction. Birch buds, leaves of eucalyptus, yarrow, nettle, shepherd's purse, serpentine rhizome and fruits of Japanese Sophora are taken in equal parts. Everything gets mixed up. Pour two tablespoons of the herbal mixture into a glass of boiling water, bring to a boil over low heat and let it brew overnight. In the morning, filter and divide the resulting solution into 3 parts. They drink during the day.

Home remedies based on traditional medicine recipes help ease the general well-being of a sick person, calm itching, pain, relieve redness and irritation.

Bullous dermatitis: video

Pemphigoid of any type and form is a serious disease that requires qualified medical treatment and control. Self-medication in such cases is unacceptable.

Pemphigoid is a skin disease that mainly affects older people. In external manifestations, the disease resembles pemphigus. Often, tense blisters are distributed on the flexor surfaces of the limbs, abdomen, and lower torso. Timely treatment of the disease will relieve a number of undesirable consequences.

What is pemphigoid

Pemphigoid is a disease, presumably of an autoimmune nature, manifested by the formation of blisters of various sizes on the skin and mucous membranes. The disease usually occurs in elderly people (over 65 years old). Pemphigoid begins with the formation on the skin of one or several thick-walled translucent blisters with a diameter of 0.5–3 cm. As the disease progresses, the number of blisters increases.

Types of disease

This group of diseases includes blistering dermatoses that are not accompanied by acantholysis, a pathological process in the spinous and granular layers of the epidermis.

Types of pemphigoid:

• scarring. This type of pemphigoid primarily affects the mucous membranes (conjunctival membrane, tonsils, uvula, soft palate). Women are more susceptible to pathology than men. People over 50 years of age are at risk. Bubbles appear in the same places, which leads to atrophic scar changes on the mucous membrane;

• bullous (Lever's pemphigoid). The main place of dislocation of vesical elements is the limbs and torso. The oral mucosa (cheeks, border of the hard and soft palate) is affected in 30% of patients. The disease affects only the top layer of skin. The rash is painful and accompanied by itching. Inside the blisters there is a clear liquid (sometimes mixed with blood);
• benign non-acantholytic pemphigus. It affects only the oral mucosa and is characterized by a chronic course;
• juvenile This is pemphigoid, which occurs in childhood;
• gestational. This is a fairly rare autoimmune pathology observed during pregnancy and in the early postpartum period. In 90% of women, the first elements of the rash appear in the navel area. Subsequently, the rash spreads to other areas of the abdomen, thighs, and hands;

• staphylococcal (pyococcal). Characterized by rapid and significant spread of bubbles. The causative agent of the disease is Staphylococcus aureus. The disease affects newborns in the first days of their life. The carrier of the infection is medical personnel and mothers of babies.

Video: all about bullous pemphigoid

Causes of the disease

To date, there is no exact data on what triggers the emergence of this disease. However, most dermatologists are inclined to the autoimmune nature of this condition. This is evidenced by the pathological reaction of the patient’s immune system to the body’s own tissues.

It is believed that factors that can provoke the disease are:

• vaccination;
• ultraviolet radiation, burns;
• radiation therapy;
• kidney transplant rejection;
• damage or severe irritation of the dermis;
• taking certain medications (Furosemide, Penicillin, Phenacetin, Captopril, Amoxicillin).

Pemphigoid is often combined with neurological diseases (Parkinson's disease, paralysis, bipolar disorder, etc.).

Factors contributing to the development of the disease are:

• smoking;
• change of climate zone;
• significant physical activity;
• stress.

Clinical signs

In the initial stage of the disease, symptoms are often nonspecific. Patients experience itching of varying intensity, sometimes there is redness on the skin, swelling and minor rashes, which can persist for a long time (up to several months). The bullous stage is characterized by the development of blisters on reddened or visually healthy skin. The blisters contain clear, sometimes blood-laced, fluid. The lesions are symmetrical in nature and are located on the mucous membranes of the eyes, nose, esophagus, and flexor surfaces of the extremities.

With bullous pemphigoid, the blisters may coalesce and affect large areas of the skin.

In the acute stage, the patient feels general malaise and loses appetite. With Lever's pemphigoid, the blisters last for several days and then spontaneously burst. In this case, erosive and ulcerative defects remain on the dermis, which heal after a few weeks.

Diagnostic measures

A disease such as pemphigoid externally manifests itself similarly to a number of other ailments (Dühring's dermatitis, true pemphigus, erythema multiforme). Therefore, to establish the correct diagnosis, it is extremely important to contact a qualified specialist. The dermatologist will perform an external examination and refer the patient for laboratory immunochemical tests. Sometimes additional consultation with an ophthalmologist and dentist is required.

In some situations (gestational, cicatricial pemphigoid), conventional diagnostics should be supplemented by sampling the contents of the blisters for further histological examination.

Diagnostic methods:

• simple light microscopy. The study excludes acantholysis - degenerative changes in the spinous layer of the skin;
• immunofluorescence microscopy. This histological method allows the detection of autoantibodies in the blood serum - an accumulation of class G immunoglobulins and complement fractions along the basement membrane of the epidermis. Using a special microscope, the glow of antibodies labeled with fluorescent dyes and associated with antigens is detected;
• immunoelectron microscopy. When carrying out this method, immunoglobulins are marked with gold, after which specialists study their location;
• immunoblotting is a method that allows you to detect autoantibodies circulating in the blood. The method is based on the separation of proteins depending on molecular weight and their further transfer using a gel to a nitrocellulose membrane. If there are antibodies against specific antigens of the pathogen, then a dark line appears on it. The material for the study is the patient's blood plasma or serum. The method is highly informative and reliable.

Table: difference between bullous pemphigoid and pemphigus vulgaris

How to get rid of pathology

The choice of treatment regimen for pemphigoid depends on a number of factors:

• general condition of the patient;
• accompanying ailments;
• prevalence of the process;
• severity of the course;
• types of illness.

Therapy is carried out by a doctor only after receiving the results of microscopic examinations.

Drug therapy

Treatment of pemphigoid comes down to:

• cytostatics and immunosuppressants (Azathioprine, Cyclosporine, Methotrexate, Chlorambucil). These drugs have powerful immunosuppressive activity and block the functioning of the immune system. Cytostatics are used for severe disease. They give a good therapeutic effect in combination with glucocorticoid hormones;
• corticosteroid hormones (Prednisolone). Drugs of this group have a pronounced anti-inflammatory, antiallergic and immunosuppressive effect, reduce the content of T-lymphocytes in the blood. The duration of treatment is determined by the severity of the disease. Corticosteroid hormones are first-line drugs for the treatment of bullous pemphigoid;
• vitamin complexes that strengthen the walls of blood vessels.

When blisters are located on the oral mucosa, rinsing with antiseptic solutions (Miramistin, Chlorhexidine, Furacilin) ​​is prescribed. To treat eye rashes, eye drops with glucocorticoids, antibacterial and antiseptic drugs (Okomistin, Levomycetin drops) are used.

Medications - photo gallery

Methotrexate is a cytostatic drug from the group of antimetabolites, folic acid antagonists, has a pronounced immunosuppressive effect Miramistin is an antiseptic that can fight various microorganisms Okomistin is an ophthalmic drug with antiseptic action that is applied topically in the form of drops Prednisolone is a synthetic glucocorticoid drug. Vitamin C has a positive effect on the condition of blood vessels.

Do I need a diet?

When treating pemphigoid, patients are prescribed a special diet and a number of restrictions are introduced. If a patient has damage to the mucous membrane of the oral cavity, esophagus, or pharynx, eating regular solid food is extremely difficult. In this case, vegetarian pureed soups, liquid porridges, fish soufflé, baked vegetables and fruits will be your salvation. A diet low in gluten is indicated.

If possible, patients should exclude sugar, smoked meats, sauces, animal fats, and reduce the consumption of dairy products to a minimum.

Hygiene

Skin with pemphigoid requires special care. To prevent further infection, blisters should be treated with antiseptics (Fukortsin, Diamond green). It is not recommended to swim if you have a blistering rash.

Brilliant green is used to treat blisters due to illness

Folk remedies

Pemphigoid is an autoimmune disease, the treatment of which requires an integrated approach. In parallel with drug therapy, decoctions, infusions and juices of medicinal plants can be used at home.

Folk remedies that are used for pemphigoid:

Patient prognosis and prevention

Timely treatment and the absence of an oncological process are the key to a favorable outcome of such a disease as pemphigoid. When following the doctor’s instructions, patients are able to achieve stable remission. During therapy, patients should adhere to a balanced diet, avoid significant physical activity and stress. The most favorable prognosis for children.

Blisters on the skin can become an open gateway for pathogenic bacteria and microbes, causing significant inflammatory processes (from suppuration to sepsis).

Localization of bullae on the conjunctiva without appropriate treatment can lead to its wrinkling, loss of mobility of the eyeball and even blindness.

It should be remembered that pemphigoid can be the beginning of an oncological process, so it is extremely important to undergo a thorough examination.

Unfortunately, there are no measures that would prevent the onset of the disease.

Pemphigoid is an autoimmune disease that requires timely drug treatment. The patient should undergo a comprehensive examination, eat a balanced diet and follow the instructions of a dermatologist (ophthalmologist, dentist).

– a chronic autoimmune skin disease that mainly affects older people. Its symptoms are similar to those of pemphigus and boil down to the formation of tense blisters on the skin of the arms, legs, and abdomen; The distribution of pathological foci is usually symmetrical. Diagnosis of bullous pemphigoid is made by examining the patient, histological examination of skin tissue in the affected areas, and immunological studies. Treatment of the disease includes immunosuppressive and cytotoxic therapy using glucocorticosteroids and cytostatic agents.

ICD-10

L12.0

General information

Treatment of bullous pemphigoid

The first-line drugs used to treat bullous pemphigoid are glucocorticosteroids - prednisolone, methylprednisolone and others. The treatment is long-term, therapy begins with high dosages of steroids, gradually reducing the dose over 6-9 months. Considering that many patients with bullous pemphigoid are elderly, it is not possible to carry out full-fledged therapy with glucocorticosteroids due to significant side effects. Often in such a situation, treatment is carried out with a combination of a reduced dose of steroids orally and local application of ointments based on them.

Treatment of bullous pemphigoid with immunosuppressive drugs - for example, cyclosporine - has good results. Cytostatic agents such as methotrexate and cyclophosphamide are used in the same way. Double filtration can significantly speed up recovery and increase the effectiveness of therapy for bullous pemphigoid. Externally, in addition to ointments with glucocorticosteroids, antiseptics (for example, aniline dyes) are used to prevent complications such as secondary infection. However, in any case, the treatment of this disease is very long and takes at least one and a half years, and even then, 15-20% of patients then experience relapses.

Forecast and prevention of bullous pemphigoid

The prognosis of classic bullous pemphigoid is uncertain in most cases. This is explained by the fact that the disease is chronic and difficult to predict, and most patients are elderly, often with other concomitant pathologies. Early high estimates of mortality from bullous pemphigoid (from 10 to 40%%) are now considered somewhat incorrect, since the calculation did not take into account age, the presence of other diseases and other factors. Childhood and adolescent forms of this pathology are in most cases successfully cured. Persons suffering from bullous pemphigoid or who have successfully undergone treatment should avoid exposure to traumatic factors on the skin - ultraviolet radiation, high or low temperatures, and mechanical injuries. This may trigger the development of a relapse of the disease.

One of the chronic autoimmune lesions of the dermis, which is often diagnosed in elderly patients, is bullous pemphigoid. The signs of this pathology are similar to those of pemphigus due to the formation of blisters on the epidermis. Let us consider in more detail the features of skin lesions, symptoms, and treatment methods.

Bullous pemphigoid is also known as Lever's disease. This autoimmune lesion of the dermis is quite rare. It is manifested by characteristic vesicular elements (bullas) that appear on the surface of the dermis. Bullous pemphigoid levera is most often diagnosed in people over 65 years of age.

Bullous pemphigoid is characterized by the presence of tense blisters that form under the epithelium due to dissection of the basement membrane. Only the upper layers of the dermis are affected.

With bullous lesions, the vesicles are localized symmetrically. They cover the following areas of the dermis:

• legs;
• stomach;
• hands.

Pemphigoid bullosa specialists may also call it senile dermatitis herpetiformis, parapemphigus. Bullous lesions of the dermis are considered chronic and are characterized by relapses. Unlike pemphigus vulgaris, bullous dermatitis occurs without acantholysis. The appearance of blisters inside the epidermis is considered a secondary process. This feature was discovered by Lever in 1953. There are very rare cases of manifestations of dermal pathology in children and adolescents (about a hundred cases in total).

Scientists have discovered a connection between bullous dermatitis and cancer pathologies. Therefore, bullous pemphigoid is sometimes considered by specialists as a paraneoplastic process. There is evidence of the development of the disease in question in people with lung, stomach, and bladder cancer.

Bullous dermatitis is more often recorded in representatives of the stronger sex. Over the years, the risk of developing the disease increases significantly. Let us consider in more detail the reasons that provoke the occurrence of bullous pemphigoid.

Causes

Experts note that bullous pemphigoid lever is autoimmune in nature. Typically, its development is triggered by a hereditary predisposition to disruptions in the autoimmune system. Experts also admit the possibility of a viral etiology of the form of dermatosis in question. At the same time, scientists have identified a number of factors under the influence of which bullous pemphigoid occurs:

• excessive irritation of the epidermis (radiation therapy, burns);
• tissue transplantation;
• injury to the dermis;
• vaccination;
• tumor.

Scientists were able to confirm the autoimmune theory of the occurrence of bullous dermatitis by finding antibodies to the basement membrane of the epidermis in the fluid of the bladder and blood of the patient.

Development of the pathological process

After any of these factors influence the immune system, a humoral cellular response occurs. It is manifested by the production of antibodies to certain epidermal cells that have become “foreign”. The autoimmune process is activated, and connections between cells in the lower layer of the skin are broken. This is how vesicles containing liquid inside are formed.

The formed bubbles merge. Dense tires appear on them, which are represented by healthy epidermal cells. The cells of the walls of the formation age and die. At the same time, the regeneration process is activated. It is represented by the formation of new cells at the bottom of the vesicle. The bladder is located between two layers of the dermis:

1. Old tire.
2. New epithelium.

Bullae may appear on the non-inflamed dermis around the vessels. With the development of the inflammatory process, areas with infiltration appear.

The fluid from the bladder contains immune cells and a certain number of leukocytes-eosinophils. With the development of any processes in the affected area, the connection between the cells of the spinous layer is maintained (there is no process of acantholysis). In other words, the destruction process is not observed. Given this feature, scientists called the pathology non-acantholytic pemphigus, Lever pemphigoid.

Characteristic signs of the disease

Usually, before the appearance of bubbles, the patient may show only mild signs of disease development. Lever's bullous pemphigoid has the following initial symptoms:

• itching with varying intensity, which is felt in the arms, lower abdomen, legs;
• redness of the skin;
• mild erythematous rash.

Only after a certain period of time do bubbles appear. Their size reaches 3 cm. In 30% of bullous pemphigoid, erosions occur in the mucous membranes of the vagina and oral cavity. A special feature of the bubbles is also the strength of their lid. The blisters that appear as a result of pathology are characterized by resistance to injury. The formations contain serous fluid, sometimes it is replaced by hemorrhagic, purulent contents.

The opening of the bladder is accompanied by the exposure of the skin to erosions, which are characterized by moisture, tenderness of the surface, and a reddish color. The epidermis in these places heals quite quickly, after the wounds there are practically no traces visible.

Additional signs of bullous pemphigoid in patients include:

• loss of appetite;
• weight loss;
• fever.

If a person suffering from bullous pemphigoid is severely exhausted, death is possible. Bullous dermatitis is characterized by a chronic course (signs of pathology gradually subside, then may appear again). In literally 15–30% of cases, doctors observed spontaneous healing of the body.

Cicatricial pemphigoid

In medical practice, the term “cicatricial pemphigoid” arose simultaneously with the term “bullous pemphigoid” due to Lever’s identification of this rare dermatosis as a separate group. This pathology is autoimmune in nature.

A feature of this form of pathology is the appearance of bullae in one place. Bubbles appear on the dermis for a long time. For this reason, scars form. Localization of the disease on the conjunctiva is considered dangerous due to the likelihood of blindness.

Women suffer from cicatricial pemphigoid 2 times more often than the stronger sex. In children, this pathology was recorded in isolated cases.

A pathological process develops due to the influence of pathological antigens (exogenous, endogenous) on the mucous membranes and dermis.

Endogenous factors are considered:

• use of dental pastes;
• taking certain medications;
• use of eye drops.

Exogenous factors are:

• eyelash hair removal;
• severe hyperinsolation;
• Bite correction using braces.

With cicatricial pemphigoid, damage to the mucous membranes of the conjunctiva, nasal cavity, mouth, esophagus, pharynx, and genitals is observed.

Doctors report damage to the mucous membranes in 70% of cases; pathology of the skin is observed less frequently (about 30–40% of cases).

The development of pathology in the conjunctival area is manifested by the following signs:

• edema;
• hyperemia;
• pain syndrome;
• photophobia;
• rashes (small blisters the size of a pinhead).

After the bubble opens, a new one forms in its place. This provokes the formation of a scar on the scar. Cicatricial pemphigoid is dangerous due to wrinkling of the conjunctiva, loss of mobility of the eyeball, cicatricial deformation of the lacrimal canals, the formation of adhesions of the conjunctival sac, and the appearance of a cataract.

Diagnostics

Lever's bullous pemphigoid can be diagnosed by examination by a dermatologist. Special studies (histological, immunological) will also be required. During the examination, the doctor examines the signs that have already appeared (erythematous rash, location of blisters, crusts on erosions, the presence of healing erosions). The medical history contains all the data after a visual examination and research.

The specialist will need a general blood test. This diagnostic method shows the following picture:

• eosinophilia (moderate);
• leukocytosis.

The immunoprecipitation reaction performed makes it possible to see IgG in peripheral blood, which can bind to antigens.

The peculiarity of histological examination is to carry out the following procedures:

• light microscopy. This diagnosis visualizes the epidermal cleft, the presence of a subepidermal bubble, which gradually turns into an intraepidermal one. The study shows severe swelling of the dermis under the lesion, leukocyte infiltration is visible (it consists of the following components: eosinophils, lymphocytes, neutrophils);
• immunofluorescence microscopy. This research method shows the accumulation of immunoglobulins of group G, the complement fraction, throughout the basement membrane of the epidermis. The concentration of these molecules in the outer region of the basement membrane is noted.

A specialist may need to conduct a differential diagnosis with the following pathologies:

• epidermolysis bullosa;
• pemphigus vulgaris;
• exudative erythema (multiform).

Basic therapeutic methods

Bullous pemphigoid is treated with medications. First-line drugs are glucocorticosteroids (methylprednisolone, prednisolone). Therapy for the pathology in question is quite lengthy and begins with large doses of steroids. Over a course of therapy of 6–9 months, the dosage of the drugs is gradually reduced.

Due to the fact that most patients with bullous pemphigoid are elderly, it is impossible to carry out full-fledged therapy with glucocorticosteroids. The use of medications in this group is dangerous due to the manifestation of a large number of side effects. Experts recommend taking small doses of steroids internally. This treatment is complemented by the use of topical ointments.

Treatment of bullous dermatitis with immunosuppressive drugs (cyclosporine and others) will be quite effective. Doctors also prescribe:

• cytostatic drugs (cyclophosphamide, methotrexate);
• antiseptics. They are necessary to prevent secondary infection and complications.

To significantly accelerate the therapeutic course, doctors recommend plasmapheresis with double infiltration. The entire therapeutic course sometimes takes up to two years. Even after treatment for bullous pemphigoid is completed, relapses may occur in 15–20% of patients.

Disease prognosis

A disease such as classic bullous pemphigoid has an uncertain prognosis. Experts explain this by the fact that bullous dermatitis is chronic and difficult to diagnose. In addition, the majority of patients are elderly people with a history of various concomitant diseases.

Previously, experts diagnosed many cases of mortality with bullous pemphigoid, but doctors did not take into account the age of the patients and the severity of concomitant pathologies. Treatment of children and adolescents is carried out successfully.

Those who have had bullous pemphigoid should avoid exposure to negative factors on the dermis:

• ultraviolet;
• mechanical injuries;
• high, low temperatures.

See also other skin diseases

Bullous pemphigoid- a rare, relatively benign bullous dermatosis that usually has a chronic course. Persistent, tense blisters form under the epidermis due to delamination of the basement membrane. Bullous pemphigoid is less common than true (acantholytic) pemphigus and usually affects older people (about 60-70 years), although it can sometimes occur in children - juvenile pemphigoid .

Etiology and pathogenesis of bullous pemphigoid

The etiology of bullous pemphigoid has not been established. The pathogenesis of the disease is autoimmune. It is based on an autoimmune reaction with the formation of autoantibodies to the proteins BP230 (BPAG1) and BP180 (BPAG2), with molecular weights of 230 and 180 kDa, respectively.

Protein BP230 Desmoplakin is an intracellular component of the hemidesmosome. Antibodies to BP230 are detected in 30-60% of patients with bullous pemphigoid and less frequently in other clinical variants.

Protein BP180- a transmembrane component of the basement membrane, consisting of type XVII collagen. Antibodies to BP180 are detected in 40-90% of patients with typical bullous pemphigoid, as well as in more rare clinical variants of pemphigoid.

It is known that autoantibodies fixed in a stripe pattern on the basement membrane activate the complement factor, which leads to the release of leukotriene B4 from mast cells and determines the chemotaxis of eosinophilic, neutrophilic granulocytes and macrophages. The proteolytic enzymes they release lead to the destruction of the upper layers of the basement membrane, the separation of the epidermis and dermis, and the formation of a subepithelial bladder. In some patients, bullous pemphigoid occurs as a paraneoplastic disease.

Clinical picture of bullous pemphigoid

Bullous pemphigoid is manifested by the development of tense blisters of varying sizes: from a few millimeters to 5-10 cm in diameter or more. Elements of a skin rash appear on apparently unchanged skin or against the background of edematous erythema and are accompanied by severe itching. The blisters have serous or serosanguineous contents due to damage to the superficial capillaries of the dermis during subepidermal detachment. When grouped, they sometimes form foci of bizarre outlines, are located against a background of erythema and resemble manifestations of dermatosis herpetiformis. In some cases, blisters and erythematous patches may precede the appearance of blisters.

The rashes are usually widespread, symmetrically located, although localized variants of bullous pemphigoid are also occasionally found. Common sites of injury are the lateral surfaces of the neck, axillary areas, inguinal folds, flexor surfaces of the limbs and the upper abdomen. Sometimes bullous pemphigoid begins with blisters on the palms and soles and resembles manifestations of erythema multiforme.

When the blisters are opened, erosions are formed without a tendency to peripheral growth, which are covered with serous and serous-sanguineous crusts, epithelialize relatively quickly, leaving pigmentation. Thus, with bullous pemphigoid, there is both true and evolutionary polymorphism of the rash.

The appearance of numerous fresh rashes is accompanied by an increase in body temperature, loss of appetite, increased itching, and a deterioration in the patient’s general well-being. The marginal Nikolsky sign can be weakly positive, while on unchanged skin next to the lesion the Nikolsky symptom is usually negative.

The oral mucosa is affected relatively rarely (about 10-20% of cases), usually with widespread skin rashes. Small tense blisters with serous or serous-hemorrhagic contents are found on the mucous membrane of the hard palate, cheeks or gums.

Unlike pemphigus vulgaris. blisters with bullous pemphigoid persist on the oral mucosa for several days due to the great depth of occurrence and thick tire. When they are opened, painful, clearly demarcated erosions without fibrinous plaque are formed, which epithelialize faster than with pemphigus. Very rarely, in addition to the skin, the mucous membrane of the pharynx, larynx, genitals, and eyes can be affected. The disease can occur chronically with exacerbations and remissions of varying durations (months, years). If untreated, the mortality rate is less than with pemphigus vulgaris (about 40%). Patients may die from secondary infection (bronchopneumonia, sepsis, etc.) or from decompensation of existing diseases. In patients with bullous pemphigoid, secondary anemia, leukocytosis with moderate eosinophilia develop, ESR increases, and the level of immunoglobulin E in the serum increases.

Diagnosis of bullous pemphigoid

In impression smears from the bottom of fresh erosion, a large number of eosinophils (20-30% or more) are found, acantholytic cells are absent. Histological examination reveals a subepidermal cavity with numerous eosinophils. The basement membrane is split and can be traced both at the base of the bladder and in its operculum. In the dermis there is swelling of the papillae and an infiltrate consisting mainly of eosinophilic granulocytes.

Using PIF, a homogeneous strip-like deposition of immunoglobulins G and Cj-complement in the basement membrane zone is detected in biopsied areas of the affected skin of patients. Using indirect IF, IgG antibodies to a protein that is part of the basement membrane are detected in the blood serum and cystic fluid in 80-90% of patients. Their titers do not correlate with the severity of the disease. Differential diagnosis is carried out with pemphigus vulgaris, bullous form of Dühring's dermatosis herpetiformis, bullous form of polymorphic exudative erythema and bullous toxiderma.

Treatment of bullous pemphigoid

In some cases, bullous pemphigoid can be a companion to cancer, so patients are examined in detail to identify malignant tumors and other concomitant diseases. Avoid medications and influences that can provoke bullous pemphigoid.

The basis of treatment for bullous pemphigoid is pathogenetic therapy with immunosuppressants: glucocorticoids alone or in combination with azathioprine or diaphenylsulfone (DDS). Prednisolone (or another GC in an equivalent dose) is prescribed in medium doses (40-60 mg per day) until a pronounced clinical effect is obtained (usually 2-3 weeks). Then the dose is gradually reduced to maintenance (10-15 mg of prednisolone per day). If no rash appears over the next 3-6 months of maintenance dose therapy, glucocorticosteroids can be completely discontinued. If there are contraindications to prednisolone, treatment with azathioprine or DDS can be tried.

External treatment of bullous pemphigoid is similar to that for pemphigus. It is rational to use antimicrobial (if localized in folds - and antifungal) drugs that are astringent and have a local anesthetic effect.

Sources:

1. Sokolovsky E.V. Skin and venereal diseases. - St. Petersburg: Foliant, 2008.

2. Thoma-Uszynski S, Uter W, et al. BP230- and BP180-specific auto-antibodies in bullous pemphigoid. J Invest Dermatol. 2004 Jun.

3. Smolin G. Foster C.S. et al. Smolin and Thoft's The Cornea: Scientific Foundations and Clinical Practice. Lippincott Williams & Wilkins, 2005.

Pemphigoid: types of disease and its manifestations, principles of therapy

Pemphigoid is a chronic benign skin disease that occurs mainly in older people. The pathology is based on the detachment of areas of the epidermis without loss of intercellular connections in one of the widest ranges. This situation arises due to various reasons in which antibodies to one’s own skin appear in the human body.

Diagnosis of pathology is carried out by a dermatologist using a combination of several instrumental techniques. Treatment includes both systemic immunosuppressive therapy and local agents. The disease responds well to therapy, but the drugs will have to be used for a long time to avoid relapse.

What is pemphigoid

This disease affects only the top layer of skin - the epidermis. To make the processes occurring during this process clear, let us briefly consider the structure of this formation.

In structure, the epidermis resembles a house of 13-16 floors, lying on hills (the dermis creates such elevations and depressions) with a very wide roof. This layer consists of four, and on the palms and soles - of five anatomically different layers:

• those that make up the “building blocks” are the germ layers. There are two of them: basal (“bricks” lie in one or two layers) and spinous (there are 10 or more rows of cells);
• The “roof” is the remaining 2 or 3 layers. They come from germ layers, but with each row they look less and less like cells (the last layer is actually scales).

The stratum spinosum is special: it is multilayered, and its cells are equipped with projections - “spines”. They are connected. In true pemphigus (pemphigus), the connection between these spines is destroyed as a result of the inflammatory process. With pemphigoid (the suffix “-oid” means “similar,” that is, “pemphigus-like” pathology), the relationship between the cells of the spinous layer remains “in force.” This is the main difference between the two pathologies, manifested by the appearance of blisters on the skin.

Under the influence of the reasons described below, immunoglobulin antibodies are formed to the membrane on which the basal layer of the epidermis lies. It is, in fact, a separator between the epidermis and dermis; it contains factors that stimulate the growth of the upper skin layer. When areas of the basement membrane come under “attack” by antibodies, a cascade of immune reactions is activated, and neutrophil cells enter here. Various enzymes are released from them, which destroy the “threads” connecting the epidermis to the dermis. The same thing happens with pemphigus, only with it there is a connection with the main tissue compatibility antigens localized on leukocytes, which does not happen with pemphigoid.

Bubbles (bullas) with bullous pemphigoid are formed as follows:

• between the processes of the cells of the lowest layer, with the help of which they communicate with each other, as a result of the autoimmune process, bubbles filled with liquid appear - vacuoles;
• In addition, swelling of the lower layer of skin - the dermis - occurs;
• gradual fusion of vacuoles, together with dermal edema, leads to the formation of large fluid cavities. The bladder cover is stretched epidermal cells, the bridges between which are preserved;
• then the epidermal cells die;
• At the same time, regenerative processes are launched in the epidermis: new cells creep from the edges of the bubble and gradually capture its bottom. This makes the blister intraepidermal.

Bullae can be located on non-inflamed skin, then they occupy a position around the vessels. If the surrounding skin becomes inflamed, pronounced infiltrates form in the dermis. The fluid filling the bullae contains many lymphocytes, histiocytes (tissue immune cells), and a small number of eosinophils (a type of white blood cell responsible for allergic manifestations).

But still, no matter what processes occur, the ligaments between the cells of the spinous layer are preserved, that is, acantholysis (their destruction) does not occur. Therefore, the disease is called a non-acantholytic process. Its second name is Lever's bullous pemphigoid.

Classification of the disease

There are several forms of this pathology:

• Actually bullous pemphigoid, also known as Lever's non-acantholytic pemphigoid. Its symptoms will be described below.
• Cicatricial pemphigoid, also called mucosynechial bullous dermatitis. The elderly age group (people over 50 years old), more often women, are susceptible to pathology. The appearance of blisters on the conjunctival membrane and oral mucosa is characteristic; Some patients experience skin rashes.
• Non-acantholytic benign pemphigus of the oral mucosa only. As the name suggests, blisters only appear in the mouth.

There is also a separate type of pemphigoid - pyococcal pemphigoid. This is an infectious disease caused by Staphylococcus aureus, which develops in children on the 3-10th day of their life. It is highly contagious. The source of infection can be the medical staff of the maternity hospital or the mother of the child who has recently had a staphylococcal infection or is a carrier of Staphylococcus aureus in the nasopharynx.

It is characterized by the rapid appearance of blisters on the baby's reddened or healthy-looking skin. Initially they appear on the torso and abdomen. The blisters initially have the size of a large pea, then they grow and can reach several centimeters in diameter and fester; they are surrounded by a pink corolla. In addition, the temperature rises and the general condition of the child may be affected. Staphylococcal pemphigoid can lead to blood poisoning, which can be fatal in severe cases.

Causes of pemphigoid

Why this disease develops is not known exactly. It is believed that pathology can occur due to:

• ultraviolet irradiation;
• taking certain medications: Penicillin, Furosemide, 5-fluorouracil, Salazopyridazine, Phenacetin, Potassium iodide, Ciprofloxacin, Amoxicillin or even Captopril;
• tumor process of any localization (therefore, when making a diagnosis of “Bullous pemphigoid”, doctors search for cancer in all possible localizations).

Symptoms of pathology

The disease has a chronic course, when a period of absence of symptoms (remission) is interspersed with a period of resumption of symptoms (exacerbation), and with each new exacerbation the pathology can spread.

Symptoms of bullous pemphigoid

The range of symptoms is quite wide. Usually this is:

• the appearance of tense blisters measuring 0.5-3 cm on reddened and swollen skin, less often on normal skin;
• in large quantities;
• itchy, less often – itchy and painful;
• localized mainly: in the folds of the skin, on the abdomen, inner thighs and shoulders, forearms. In a third of cases, they appear in the size of 0.5-2 cm on the oral mucosa: on the cheeks, on the border between the hard and soft palate, on the gums;
• most often the bubbles are located symmetrically;
• have transparent contents, which over time may become purulent (yellow or white) or bloody;
• at the same time, like the bullae, urticaria-like elements of the rash appear, having a red or pink-red color. Such blisters are especially noticeable when the redness of the skin on which the blisters are located subsides;
• after the blister opens, a moist area of ​​pink-red color remains, which quickly heals with or without the formation of crusts;
• the general condition of most people does not suffer: consciousness is not depressed, there is no fever, weakness, nausea or vomiting. Depleted patients and the elderly may experience loss of appetite and weakness; they may lose weight.

In the initial stages of the disease there may be no blisters, only crusts, eczema-like polymorphic elements or blisters like urticaria. The rash may be accompanied by itching of varying intensity, which is difficult to treat. Subsequent exacerbation occurs with the usual symptoms, if this is the classic form of pemphigoid, or with the repetition of the same symptoms, with atypical forms.

Manifestations of cicatricial pemphigoid

The rash can be found on the soft palate, buccal mucosa, uvula and tonsils, while the oral mucosa is red and swollen, but may not be changed. Sometimes eruptive elements appear on the lips, on the conjunctiva of the eyes, and, developing on the skin, are localized on the face, in folds (especially on the thigh), and scalp. The disease can also affect internal organs.

The rash is tense blisters, the contents of which are clear or bloody. After opening them, deep red erosions are visible.

A characteristic sign of cicatrizing pemphigoid is the appearance of blisters constantly in the same places, which contributes to the development of scars there. This scarring in the lip area makes it difficult to open the mouth. Activated on the conjunctival membrane of the eye, the cicatricial process leads to its wrinkling, limitation of movements of the eyeball, and disruption of the patency of the lacrimal canals. Ocular localization can also cause ulcers to appear on the cornea, causing it to become cloudy and only allow a person to perceive light.

The cutaneous localization of this form of pathology causes the appearance of scars located below the level of the underlying skin. Developing on the internal organs, the disease can be complicated by deterioration of the patency of the larynx, esophagus, urethra, vagina or anus.

Diagnostics

The diagnosis of Bullous Pemphigoid or Cicatricial Pemphigoid is made on the following grounds.

1. Inspection: pemphigoid has a characteristic localization and a typical high density of blisters.

2. Skin biopsy, according to which the following is carried out:

• conventional microscopy: acantholysis is excluded (loss of communications between the spines of cells of the spinous layer);
• immunofluorescence microscopy, which allows the dermatologist to see the glow of the skin in the area not of the spinous layer, but of the basement membrane;
• immunoelectron microscopy: gold labeling of immunoglobulins is used, after which its location is studied;
• method of immunoblotting and immunoprecipitation.

Therapy

Treatment of bullous pemphigoid consists of introducing drugs into the body that will block the functioning of the immune system - these are:

1. Glucocorticoid hormones: prednisolone, dexamethasone, starting with small doses (30-40 mg of prednisolone per day).
2. Cytostatics (similar drugs are also used in chemotherapy of cancer tumors): Azathioprine, Cytoxan, Methotrexate.

In case of severe pathology, the simultaneous use of both glucocorticosteroid and cytostatic drugs is recommended for the first 2 weeks.

To increase the effectiveness of the above means, the following are used:

3. systemic enzymes: Phlogenzyme, Wobenzym;
4. vitamins necessary to strengthen the vascular wall: vitamin P, C, nicotinamide;
5. immunotherapy drugs: Rituximab.

If it is proven that bullous pemphigoid has arisen as a result of the development of a malignant tumor in the body, antitumor therapy is carried out. It will depend on the location of the tumor, its degree of malignancy, and the stage of the disease. Can be surgical, radiation, medication (chemotherapy, targeted therapy).

Local treatment depends on the location of the lesions:

6. If they are on the skin, they are treated with ointments based on glucocorticoid hormones: prednisolone, hydrocortisone, Aklovate, Afloderm, Topicort, Oxycort.
7. When the blisters are located in the eye, local therapy is prescribed by an ophthalmologist. These are eye drops with glucocorticoids (dexamethasone drops), antibacterial and antiseptic agents (Ocomistin, chloramphenicol drops) - for the prevention of purulent complications. If, after damage to the blisters, erosions on the conjunctival membrane do not epithelialize, the drug Korneregel is prescribed.
8. If the eruptive elements are located on the oral mucosa, rinses with antiseptics are prescribed: an aqueous solution of furatsilin, chlorhexidine, miramistin.

Skin care for bullous pemphigoid includes treating the blisters with antiseptics such as brilliant green solution, methylene blue or fucorcin. These medications will dry out the bullae and prevent them from becoming infected. If you have a blistering rash, swimming is not recommended. Hygiene is carried out with gauze wipes soaked in aqueous solutions of antiseptics: chlorhexidine, furatsillin. This should be done with blotting movements.

What to do with non-healing erosions with pemphigoid?

This happens either due to infection or poor regenerative abilities. In the first case, instead of single-component ointments containing only glucocorticoids, combination products with hormones and antibiotics are prescribed: Pimafucort, Imakort, Aurobin.

Poor regenerative abilities require further clarification of the reasons: it could be diabetes mellitus or vascular pathology. Then, if appropriate treatment is prescribed, the erosions will heal. Until the etiology is clarified or if it remains unknown, and in addition to treatment of the causative disease, dexpanthenol in the form of Bepanten cream or methyluracil in the form of a gel-like drug Levomekol is prescribed.

Do you need a diet?

A diet for bullous pemphigoid is needed in order not to expose the body, in which the immune system is strained, to additional allergenic influences. It consists of observing the following rules:

9. there should be enough vegetables in the diet;
10. replace meat with fish;
11. dairy products – minimum;
12. You should try to completely eliminate sugar;
13. trans fats - margarine, mayonnaise, sauces, fried foods, lard, ham, sausages - should be excluded.

What can you eat?

Greens, vegetables, fruits, sea fish, liver, whole grain bread, cereals, green tea, boiled or baked lean meat (chicken, veal), soups with a second broth or vegetarian.

Bullous pemphigoid: causes, symptoms, treatment

Bullous pemphigoid is an autoimmune skin disease that causes chronic blistering in older patients. Diagnosis is made using a biopsy. First of all, glucocorticoids are used in treatment. Many patients require long-term maintenance therapy, during which various medications may be used.

In bullous pemphigoid, antibodies are directed against the basement membrane and cause separation of the epidermis from the dermis. Bullous pemphigoid must be distinguished from pemphigus vulgaris, which is a more serious disease.

Symptoms and signs

Characteristic tense blisters appear on normal or reddened skin. Nikolsky's symptom is negative. Swollen or ring-shaped, dark red lesions with or without blistering may appear. There is often itching without any other symptoms. Lesions of the oral mucosa occur in 1/3 of patients, but usually heal quickly.

It is necessary to distinguish ordinary pemphigus from pemphigoid, IgA linear dermatosis, erythema multiforme, rashes caused by the use of drugs, benign pemphigoid of the mucous membranes, dermatosis herpetiformis, congenital epidermolysis bullosa. Diagnosis requires a skin biopsy and determination of antibody titers in the blood serum.

Prognosis and treatment

The prognosis is usually good and the disease is usually curable within months or years, but can be fatal, especially in older patients.

For mild forms of the disease, local glucocorticoids can be used in treatment. Patients with more severe forms of the disease are prescribed prednisone 60-80 mg orally once a day, after a few weeks reducing the dose to a maintenance dose of 10-20 mg once a day. In most patients, remission occurs within 2-10 months. The occurrence of isolated new rashes in elderly patients does not require an increase in dosage.

The disease is sometimes treatable with tetracycline and nicotinamide. Dapsone, sulfapyridine, erythromycin and tetracycline may also be used as they have anti-inflammatory effects. Most patients do not require immunosuppressants, but azathioprine, cyclophosphamide, cyclosporine, or plasmapheresis may be used.

PEMFIGOID

O. L. Ivanov, A. N. Lvov

"Dermatologist's Handbook"

PEMPHIGOID (synonym: non-acantholytic pemphigus) is a benign chronic skin disease, the primary element of which is a bubble that forms subepidermally without signs of acantholysis.

Nikolsky's symptom is negative in all modifications. The blisters usually regress without a trace and are much less likely to leave scars. This circumstance gave reason to distinguish between two variants of pemphigoid - bullous and cicatricial.

Etiology pemphigoid is unknown. In some cases it may be paraneoplastic in nature. The autoallergic nature of the disease is most substantiated: autoantibodies to the basement membrane of the epidermis (usually IgG, less often IgA and other classes) were detected.

Histologically in the early stages of pemphigoid, the formation of subepidermal microvacuoles is detected. Their fusion leads to the formation of blisters that separate the epidermis from the dermis. Subsequently, the epidermis, which makes up the covering of the bladder, becomes necrotic and destroyed, with the exception of the stratum corneum. After the formation of blisters, re-epithelialization of their bottom occurs, and they can be located in the dermis up to the subcorneal localization. There are no signs of acantholysis. With cicatricial pemphigoid, fibrosis of the upper layers of the dermis and a decrease in elastic tissue are noted.

Pemphigoid bullous has many synonyms:

14. chronic pemphigus vulgaris,
15. pemphigus vulgaris benign,
16. parapemphigus,
17. senile dermatitis herpetiformis,
18. dermatitis herpetiformis bullous.

They reflect the similarity of bullous pemphigoid with true (acantholytic) pemphigus and dermatitis herpetiformis. Moreover, the similarity with pemphigus is clinical due to blistering rashes, and with dermatitis herpetiformis - pathohistological due to the subepidermal formation of blisters.

It is distinguished from pemphigus by a benign chronic course and subepidermal formation of blisters, and from dermatitis herpetiformis by a monomorphic bullous rash; The predominant affection of elderly people (over 60 years of age) distinguishes bullous pemphigoid from pemphigus. and from dermatitis herpetiformis. Damage to the mucous membranes, unlike true pemphigus, is not inevitable, although it is not exceptionally rare.

The disease begins with the appearance of blisters against the background of erythematous-edematous spots, less often on apparently unchanged skin. Bubbles of medium size (from a pea to a bean), hemispherical in shape, with a dense, smooth and tense lining, serous or serous-hemorrhagic contents. Due to the dense covering, they are more persistent than the blisters of true pemphigus.

Erosions after their opening do not tend to grow peripherally and quickly epithelialize. When the contents of the blisters and erosion discharge dry out, yellowish-brown crusts of various sizes and thicknesses are formed. When they are rejected, pink-red spots covered with scales are exposed. The predominant localization is the lower half of the abdomen, inguinal folds, axillary fossae and flexor surfaces of the arms and legs. Damage to the mucous membranes is observed in approximately 20-40% of patients with bullous pemphigoid and occurs, with rare exceptions, secondarily. As the process progresses, and sometimes from the very beginning, blisters spread across the skin until a generalized and even universal rash forms. Subjectively - often itching of varying intensity, burning and soreness.

Relapses of pemphigoid are often caused by UV rays, both natural and artificial.

Over time, the severity of the disease gradually weakens, but bullous pemphigoid is a potentially serious disease that cannot exclude death.

Pemphigoid cicatricial. like bullous, it has many synonyms:

19. benign pemphigoid of the mucous membranes,
20. chronic mucocutaneous pemphigoid dermatitis,
21. pemphigus eye (conjunctiva),
22. pemphigoid mucosynechial.

The essence of the disease is most fully reflected by the term “bullous atrophying mucosynechial dermatitis”, emphasizing its main clinical feature - the outcome of blisters into scars, adhesions, and atrophy. Occurs 2 times more often in women than in men; older people get sick.

Cicatricial pemphigoid is a disease of the mucous membranes: in about a third of patients, the skin is secondarily involved in the process. The mucous membranes of the mouth and conjunctiva of the eyes are most often affected. In the oral cavity, tense blisters with a diameter of 0.2 to 1.5 cm appear on an externally unchanged mucous membrane or on an erythematous background; their contents are serous, rarely hemorrhagic. Erosion that occurs when the covering of the blisters ruptures is not prone to peripheral growth, does not bleed, their surface is free from any layers, they are not bordered by exfoliating epithelium; low pain. Salivation and swelling of the mucous membranes are insignificant.

Cicatricial adhesive and atrophic changes occur within 3 years after the onset of the disease.

It is distinguished by great originality cicatricial pemphigoid in the eye area. Already in the early stages, signs of scarring may appear in the form of small adhesions between the conjunctiva of the eyelids and the eyeball or between the upper and lower eyelids. As scarring increases, the vaults of the conjunctival cavity decrease until complete obliteration. A peculiar consequence of long-term scarring pemphigoid is the so-called sculpted eyes, in which the cornea is completely covered with a cloudy membrane, allowing only the perception of light.

On the skin there are single blisters, rarely generalized; occur on apparently healthy or erythematous skin and are persistent; erosions after them slowly heal with the formation of atrophic scars, leading to baldness on the scalp. With cicatricial pemphigoid, various functional and morphological disorders of the trachea and larynx, esophagus, vagina and anus, urethra, etc. can also occur.

Diagnosis of bullous and cicatricial pemphigoid is based on:

23. clinical and histological data
24. results of indirect and direct immunofluorescent studies.

Differentiate pemphigoid especially difficult from pemphigus vulgaris. especially in its initial stages, when acantholytic cells are often not detected, and Nikolsky’s symptom is negative. The final diagnosis is helped by the results of histological (subepidermal rather than intraepidermal location of the bladder) and immunofluorescence (luminescence in the area of ​​the basement membrane, and not in the area of ​​the spinous layer) studies.

Pemphigoid is also differentiated from:

Scarring pemphigoid is also differentiated from:

Corticosteroid hormones. The initial dose is 40-80 mg of prednisolone per day; for cicatricial pemphigoid with eye damage, higher doses may be required.

The duration of treatment and the rate of reduction in the daily dose are determined by the severity of the disease.

Cytostatics are also used, as in true pemphigus, and sulfone drugs, as in dermatitis herpetiformis.

If the disease is paraneoplastic, antitumor therapy is used.