Bullous pemphigoid. Bullous pemphigoid: what is it, symptoms and treatment. Treatment with folk remedies

Bullous pemphigoid (L12.0)

Dermatovenereology

general information

Short description

RUSSIAN SOCIETY OF DERMATOVENEROLOGISTS AND COSMETOLOGISTS

Moscow - 2015

Code according to the International Classification of Diseases ICD-10
L12.0

Definition
bullous pemphigoid ( bullous pemphigoid) is an autoimmune skin disease caused by the production of autoantibodies to hemidesmosome components (BP180 and BP230 antigens) and characterized by the formation of subepidermal blisters.

Classification

There is no generally accepted classification.

Etiology and pathogenesis

In most cases, the development of bullous pemphigoid is not associated with any provoking factor. In some patients with bullous pemphigoid, the appearance of rashes is due to medication, exposure to physical factors, and viral infections.

Drugs that may be associated with the development of bullous pemphigoid are penicillamine, penicillins and cephalosporins, captopril and other angiotensin-converting enzyme inhibitors; furosemide, aspirin and other non-steroidal anti-inflammatory drugs, nifedipine. There are known cases of the development of bullous pemphigoid after the introduction of the influenza vaccine, antitetanus toxoid. The development of bullous pemphigoid after exposure to physical factors - ultraviolet radiation, radiation therapy, thermal and electrical burns, after surgical procedures is described. It is assumed that viral infections (hepatitis B and C viruses, cytomegalovirus, Epstein-Barr virus) can contribute to the development of bullous pemphigoid.

The development of bullous pemphigoid is caused by the production of IgG autoantibodies to BP180 (collagen type XVII) and BP230 proteins, which are part of hemidesmosomes, which are a structural component of the basement membrane of the skin.

According to the Federal Statistical Surveillance, the incidence of bullous pemphigoid in the Russian Federation in 2014 was 1.1 cases per 100,000 adults (aged 18 years and older), and the prevalence was 2.6 cases per 100,000 adults. Mostly the elderly are affected. Among people over the age of 80, the incidence of bullous pemphigoid reaches 15-33 cases per 100,000 of the corresponding population per year.

Clinical picture

Symptoms, course

Skin lesions in bullous pemphigoid may be localized or generalized. Rashes are more often localized on the limbs, abdomen, inguinal-femoral folds, on the inner surface of the thighs. Rashes in patients with bullous pemphigoid may be polymorphic. The disease usually begins with the appearance of erythematous, papular and / or urticaria-like rashes, accompanied by itching. These rashes can exist for several months, after which blisters appear. Bubbles have a tense, dense cover, round or oval shape, serous or serous-hemorrhagic contents, are located on an erythematous background or on apparently unchanged skin. Formed on the site of erosion bubbles, in the absence of secondary infection, quickly epithelialize, not prone to peripheral growth. Nikolsky's symptom is negative. Mucous membranes are affected in 10-25% of patients. The disease is characterized by a chronic relapsing course.

The severity of bullous pemphigoid is determined by the number of vesicular elements that appear. Bullous pemphigoid is defined as severe when more than 10 blisters appear per day for 3 days in a row, as mild - when 10 or fewer blisters appear per day.

Diagnostics

Diagnosis of bullous pemphigoid is based on the identification of clinical signs of the disease and the detection of IgG antibodies to the proteins of the skin basement membrane components:
At histological examination A skin biopsy specimen with a fresh bladder reveals a subepidermal cavity with a superficial infiltrate in the dermis, consisting of lymphocytes, histiocytes and eosinophils, which does not always make it possible to distinguish bullous pemphigoid from other diseases with a subepidermal location of the bladder (Dühring's dermatitis herpetiformis, acquired epidermolysis bullosa).
To detect IgG to proteins components of the basement membrane of the skin is carried out immunohistochemical study a biopsy of apparently unaffected skin of a patient, in which a linear deposition of IgG and / or C3 of the complement component in the basement membrane region is detected. If necessary, differential diagnosis with acquired epidermolysis bullosa is carried out additional immunofluorescent study skin biopsy, previously split by keeping in 1M sodium chloride solution for 1 day. This study reveals the deposition of IgG in the upper part (cover) of the cavity formed in the zone of the dermo-epidermal junction.

Differential Diagnosis

The disease should be differentiated with the bullous form of Duhring's dermatitis herpetiformis, exudative erythema multiforme, pemphigus vulgaris, bullous toxidermia, acquired epidermolysis bullosa.

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Treatment

Purpose of treatment
- achieving remission.

General notes on therapy
When prescribing and conducting therapy for patients with bullous pemphigoid, the following should be considered:
1) Restrictions on the use of a number of drugs in elderly patients.
2) Possible concomitant diseases of the patient (diabetes mellitus, arterial hypertension, coronary heart disease, neurological diseases).
3) Adverse events associated with systemic therapy and topical therapy.
During treatment with systemic glucocorticosteroids, it is necessary to measure blood pressure to monitor the state of the cardiovascular system and control blood glucose levels.
During therapy with cytostatics, the content of hemoglobin and erythrocytes, leukocytes and platelets in peripheral blood, indicators of liver and kidney function, and general urinalysis should be monitored. When conducting therapy with systemic glucocorticosteroid drugs and immunosuppressants, it is also necessary to timely identify signs of infectious diseases and complications.

Treatment regimens

For mild bullous pemphigoid:
- clobetasol dipropionate 0.05% 1 time per day externally on the lesions (B) .
15 days after reaching the clinical effect (cessation of the appearance of new rashes and itching, the beginning of epithelialization of erosions), the amount of topical glucocorticosteroid drug applied is gradually reduced (D).
In the absence of a clinical effect from therapy with a topical glucocorticosteroid drug for 1-3 weeks:
- oral prednisolone at a dose of 0.5 mg per kg of body weight per day (C). Upon reaching the clinical effect, the dose of prednisolone is gradually reduced to 0.1 mg per kg of body weight per day. The duration of therapy is 4-12 months.

For severe bullous pemphigoid:
- clobetasol dipropionate 0.05% (B) externally 1 time per day on the lesions. 15 days after reaching the clinical effect (cessation of the appearance of new rashes and itching, the beginning of epithelialization of erosions), the amount of topical glucocortisteroid drug applied is gradually reduced (D).
+
- oral prednisone 0.5-0.75 mg per kg of body weight, depending on the severity of the condition. When prescribed in a daily dose of less than 0.5 mg per kg of body weight, the effectiveness of prednisolone is insufficient. Increasing the dose of prednisolone above 0.75 mg/kg of body weight does not lead to an increase in the effectiveness of therapy. A gradual decrease in the dose of systemic corticosteroid is started 15 days after the clinical effect of therapy is achieved - the cessation of the appearance of new rashes and itching, the onset of epithelization of erosions, and continues for 4-6 months to a maintenance dose of 0.1 mg / kg / day. If the patient is in clinical remission within 3-6 months, treatment may be discontinued (D) .
In case of relapse, the dose of the corticosteroid is increased to the original level.

If it is necessary to reduce the dose of systemic corticosteroids, the following are prescribed:
- Plasmapheresis 8 treatments over 4 weeks in combination with oral prednisolone at a daily dose of 0.5 mg per kg of body weight (C)
or
- azathioprine 2 mg/kg/day for 3-4 weeks in combination with prednisolone 0.5 mg/kg/day (C) . The administration of azathioprine 100-150 mg orally per day in combination with prednisolone 1 mg/kg body weight per day does not lead to an increase in the effectiveness of the treatment of bullous pemphigoid compared with prednisolone 1 mg per kg body weight per day, but causes an increase in the number of undesirable therapy-related events (C) .

Hospitalization

Indications for hospitalization

Severe course of bullous pemphigoid requiring systemic therapy;
- lack of effect from ongoing treatment with topical corticosteroids on an outpatient basis;
- the presence of secondary infection in the lesions.

Information

Sources and literature

1. Clinical recommendations of the Russian Society of Dermatovenerologists and Cosmetologists
   1. 1. Kirtschig G., Middleton P., Bennett C. et al. Interventions for bullous pemphigoid. Cochrane Database Syst Rev 2010; 10: CD002292. 2. Parker S.R., Dyson S., Brisman S. et al. Mortality of bullous pemphigoid: an evaluation of 223 patients and comparison with the mortality in the general population in the United States. J Am Acad Dermatol 2008; 59(4): 582–588. 3. Schmidt E., Zillikens D. Pemphigoid diseases. Lancet 2013; 381:320–332. 4. Lo Schiavo A., Ruocco E., Brancaccio G. et al. Bullous pemphigoid: Etiology, pathogenesis, and inducing factors: Facts and controversies. Clin Dermatol 2013; 31:391–399. 5. Joly P., Roujeau J.C., Benichou J. et al. A comparison of two regimens of topical corticosteroids in the treatment of patients with bullous pemphigoid: a multicenter randomized study. J Invest Dermatol 2009; 129(7): 1681–1687. 6. Feliciani C., Joly P., Jonkman M.F. et al. Management of bullous pemphigoid: the European Dermatology Forum consensus in collaboration with the European Academy of Dermatology and Venereology. Br J Dermatol 2015; 172:867–877. 7. Murrell D.F., Daniel B.S., Joly P. et al. Definitions and outcome measures for bullous pemphigoid: recommendations by an international panel of experts. J Am Acad Dermatol 2012; 66:479–485. 8. Joly P., Roujeau J.C., Benichou J. et al. A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med 2002; 346(5): 321–327. 9. Roujeau J.C., Guillaume J.C., Morel P. et al. Plasma exchange in bullous pemphigoid. Lancet 1984; 2 (8401): 486–488. 10. Morel P., Guillaume J.C. Treatment of bullous pemphigoid with prednisolone only: 0.75 mg/kg/day versus 1.25 mg/kg/day. A multicenter randomized study. Ann Dermatol Venereol 1984; 111(10): 925–928. 11. Beissert S., Werfel T., Frieling U. et al. A comparison of oral methylprednisolone plus azathioprine or mycophenolate mofetil for the treatment of bullous pemphigoid. Arch Dermatol 2007; 143(12): 1536–1542. 12. Guillaume J.C., Vaillant L., Bernard P. et al. Controlled trial of azathioprine and plasma exchange in addition to prednisolone in the treatment of bullous pemphigoid. Arch Dermatol 1993; 129(1): 49–53. 13. Du-Thanh A., Merlet S., Maillard H. et al. Combined treatment with low-dose methotrexate and initial short-term superpotent topical steroids in bullous pemphigoid: an open, multicentre, retrospective study. Br J Dermatol 2011; 165(6): 1337–1343. 14. Heilborn J.D., Ståhle-Bäckdahl M., Albertioni F. et al. Low-dose oral pulse methotrexate as monotherapy in elderly patients with bullous pemphigoid. J Am Acad Dermatol 1999; 40:741–749. 15. Dereure O., Bessis D., Guillot B., Guilhou J.J. Treatment of bullous pemphigoid by low-dose methotrexate associated with short-term potent topical steroids: an open prospective study of 18 cases. Arch Dermatol 2002; 138:1255–1256. 16. Gual A., Iranzo P., Mascaro J.M. Treatment of bullous pemphigoid with low-dose oral cyclophosphamide: a case series of 20 patients. J Eur Acad Dermatol Venereol 2014; 28:814–818. 17. Le Roux-Villet C., Prost-Squarcioni C., Oro S. et al. Role of the nurse in care of bullous pemphigoid. Rev Infirm 2010; 160:38–40. 18. Venning V.A., Taghipour K., Mohd Mustapa M.F. et al. British Association of Dermatologists’ guidelines for the management of bullous pemphigoid 2012. Br J Dermatol 2012; 167:1200-1214. 19. Milyavsky A.I., Krivoshein Yu.S., Logadyr T.A., Vintserskaya G.A. The effectiveness of miramistin in dermatovenereology. Vestn. Dermatol. Venerol. 1996; (2). 67–69. 20. Privolnev V.V., Karakulina E.V. Basic principles of local treatment of wounds and wound infection. Klin microbiol antimicrobial chemoter 2011, 13, (3): 214–222.

Information

The personal composition of the working group for the preparation of federal clinical guidelines for the profile "Dermatovenereology", section "Bullous pemphigoid":
1. Karamova Arfenya Eduardovna - Head of the Department of Dermatology, Federal State Budgetary Institution "State Scientific Center for Dermatovenereology and Cosmetology" of the Ministry of Health of Russia, Candidate of Medical Sciences, Moscow
2. Chikin Vadim Viktorovich - Senior Researcher, Department of Dermatology, Federal State Budgetary Institution "State Scientific Center for Dermatovenereology and Cosmetology" of the Ministry of Health of Russia, Candidate of Medical Sciences, Moscow
3. Lyudmila Fedorovna Znamenskaya - Leading Researcher of the Department of Dermatology, Federal State Budgetary Institution "State Scientific Center for Dermatovenereology and Cosmetology" of the Ministry of Health of Russia, Doctor of Medical Sciences, Moscow

METHODOLOGY

Methods used to collect/select evidence:

Bullous pemphigoid is a dermatological disease associated with a malfunction of the immune system due to tissue exposure to certain autoimmune bodies. Most often, it develops in older people and is similar in symptoms to pemphigus: symmetrically located tense blisters with a clear liquid inside form on the skin of the limbs and abdomen. To diagnose the disease, immunological and histological studies are required, and cytostatic and glucocorticosteroid agents, vitamins and diet are prescribed for treatment.

Features of bullous pemphigoid

Lever's bullous pemphigoid is a chronic skin disease that is accompanied by numerous rashes and itching. On the mucous membranes, a rash appears extremely rarely. Correct diagnosis allows biopsy, immunofluorescent examination of the skin and blood serum. In addition to glucocorticosteroids, in most cases, long-term maintenance treatment with immunosuppressants is necessary.

The risk group includes men over 60 years of age, women are less likely to get sick, very rarely children. Tension blisters (bulls) on the surface of the skin are formed due to the fusion of autoimmune IgG antibodies with BPAg1 or BPAg2 antigens. Depending on the distribution of the rash, two forms of bullous pemphigoid are distinguished - localized and generalized. According to the nature of the course and symptoms, the disease is divided into typical and atypical.

An atypical form is pemphigoid:

• Dishydrosiform (similar to atopic eczema, the rash is localized on the soles and palms).
• Nodular (multiple nodular formations on the skin, itching).
• Vegetative (numerous plaques in the folds of the skin).
• Vesicular (groups of small vesicles - vesicles).
• Nodular (nodules on the skin).
• Erythrodermic (symptoms are very similar to erythroderma).
• Eczematous (symptoms are similar to eczema).

The term "pemphigus" for a long time meant any kind of blistering rash. It was not until 1953 that Dr. Lever identified the clinical and histological features characteristic of bullous pemphigoid. Ten years later, scientists found that antibodies circulating in the affected tissues act on the basement membrane of the skin. This led to the conclusion that it is they that cause the exfoliation of the epidermis, as a result of which blisters form on the skin.

Pemphigoid develops in two stages:

• Premonitory. Lasts from several months to several years, accompanied by itching and nonspecific rash. At this stage, it is difficult to make a correct diagnosis, so the disease is often confused with cholinergic urticaria, Dühring's dermatitis, prurigo, chronic eczema, and various types of itchy dermatoses.
• bullous. Bubbles appear on the skin, the itching does not decrease. At this stage, the disease is easy to diagnose.

Sometimes pemphigoid is accompanied by headache and fever, as in chicken pox and other infectious diseases. Elderly patients often lose their appetite, feel weak. The disease proceeds for a long time, periods of regression alternate with periods of exacerbations. The probability of getting sick before the age of 60 is very small, after 60 - 8 cases per million, after 90 years - 250 per million. Some researchers have linked pemphigoid to a number of vaccines and organ transplants. But whether the disease is transmitted in this way has not yet been proven. What Lever's disease looks like at different stages can be seen in the photos below.

Symptoms of bullous pemphigoid

Most often, the classic form of bullous pemphigoid is diagnosed. As a rule, the rash appears on the trunk and limbs. Sometimes it can be found in large natural folds of the skin, on the face and scalp. The rashes are multiple, the foci are symmetrical. These are vesicles (vesicles, bullae) with a tense surface, filled with a clear liquid, less often with pus. In some cases, they occur on healthy skin, but more often on reddened. The characteristic rash may be diluted with papules and urticaria, as in sorcaidosis.

After a few days, the bubbles spontaneously burst, in their place, erosions and ulcers form. However, they heal quite quickly, so they do not tighten with crusts.
A rash on the oral mucosa appears only in 20% of patients, anyway, then bubbles appear on the trunk and limbs. Rash on the conjunctiva, mucous membranes of the nasopharynx and genital organs - isolated cases.

In places where the rash is localized, a feeling of itching appears, sometimes patients complain of headache, loss of appetite, and general weakness. Bullous pemphigoid refers to chronic diseases, its course is a series of regressions and relapses.

Causes of bullous pemphigoid

According to its etiology, the disease has an immunological origin. Autoimmune antibodies that affect the epidermis allow us to assert this; they are found in 100% of patients. The presence of these antibodies is signaled by the antiC3 conjugate in the immunofluorescence reaction. They act on the transmembrane protein (collagen) and cytoplasmic protein (BP230), which are responsible for the integrity of the epithelium.

Factors provoking Lever's bullous pemphigoid are:

• Reception of penicillamine, penicillin, sulfasalazine, spironolactone, furosemide, neuroleptics and some other drugs.
• Ultraviolet rays combined with anthralin, radiation therapy in the treatment of breast cancer.
• Multiple sclerosis, lichen planus, ulcerative colitis, rheumatoid arthritis, diabetes, some other chronic diseases.

bullous pemphigoid in children

Bullous pemphigoid in children is diagnosed extremely rarely, therefore it is not considered as a separate group of diseases. For treatment in this case, the same methods are used as for adults, but a more careful and balanced approach is required.

Diagnosis of bullous pemphigoid

Diagnosis of Lever's bullous pemphigoid is based on the results of clinical blood tests and laboratory tests of material taken from the affected areas.

After questioning and examining the patient, the doctor prescribes:

• An exfoliation test (Nikolsky's symptom) - with a slight mechanical effect on the damaged areas, it is negative, with pemphigus - positive.
• Electronic and light microscopic examination - the fibrin network of the bladder cavity (bulls), monoclear infiltrates (seals), epidermal fissure, eosinophilic thickening of the skin are studied.
• Immunofluorescence (direct and indirect) - for circulating antibodies to the basement epidermal membrane, detection of C3 and (or) IgG1, IgG4 along it, as well as the absence of acantholysis.
• Immune microscopic examination - using electric current and gold.
• Immunochemical study by immunoblotting of keratinocyte extract or immunoprecipitation - to determine class G autoantibodies.
• Analysis of eosinophils - for their number in the blood and the contents of the vesicles.
• Iodine test (Yadasson test) - the test is considered positive if after taking a 5% solution of potassium iodide and applying an ointment with 50% of this substance to the damaged skin, an exacerbated reaction of the epidermis is observed.

If the disease is severe, potassium iodide is not taken orally because of the danger of severe exacerbation.

Lever's bullous pemphigoid is differentiated from:

• Cholinergic urticaria.
• Atopic eczema.
• Sorcaidosis.
• Dermatitis Duhring.
• Prurigo.
• Chronic eczema.
• Erythroderma.
• Multimorphic erythema.
• Bullous toxicoderma.
• Neacantholytic pemphigus.
• True acantholytic pemphigus.

Treatment of bullous pemphigoid

The basis of the treatment of bullous pemphigoid is hormonal drugs. Assign average doses of drugs in this group with a gradual decrease as you recover.

In more severe cases are assigned:

• Cytostatic agents.
• Antihistamines.
• Vitamins.

To relieve itching and heal the rash, external agents are used - special creams and ointments. If the doctor allows, you can turn to traditional medicine.

Folk remedies:

• Eleutherococcus tincture - take 30 drops 2 times a day.
• Herbal collection - mix in equal proportions yarrow, shepherd's purse, nettle leaves, serpentine root, birch buds, eucalyptus leaves and Japanese Sophora fruits. 2 tbsp. spoons of the mixture pour 200 ml of boiling water, leave for 10-12 hours. Strain, take 70 ml three times a day.
• Nettle or aloe - squeeze the juice from the leaves. Soak a bandage in it and apply it to the rash. From above, cover with a film for compresses, fix with a plaster or bandage.

Complications of bullous pemphigoid

With improper treatment of bullous pemphigoid or its absence, there is a high probability of attaching a secondary bacterial or viral infection. As a result - violations of the immune system, in severe cases - sepsis and death. Whether bullous pemphigoid can degenerate into cancerous tumors is still unclear.

Prevention of bullous pemphigoid

There are no primary preventive measures to prevent Lever's bullous pemphigoid, and in order to prolong the period of remission and avoid exacerbation, the patient must follow a gluten-free diet, avoid exposure to sunlight on the skin, mechanical and thermal injuries.

The prognosis of treatment is uncertain, since this is a chronic, difficult-to-predict disease, and most patients are elderly people with various comorbidities. According to some reports, the mortality rate is 30%, but this does not take into account aggravating factors and other diseases. In children and adolescents, Lever's syndrome is easily cured.

Do not forget that bullous pemphigoid often develops as a secondary disease against the background of other pathological processes. It is considered a cancer marker. Therefore, when the first signs appear, you should consult a doctor as soon as possible. He will diagnose, prescribe treatment and send you for a comprehensive examination that can dispel suspicions of cancer. If the diagnosis is disappointing, with a high degree of probability it can be argued that this is squamous cell skin cancer.

is a chronic autoimmune skin disease that mainly affects older people. Its symptoms are similar to those of pemphigus and are reduced to the formation of tense blisters on the skin of the arms, legs, and abdomen; the distribution of pathological foci is usually symmetrical. Bullous pemphigoid is diagnosed by examining the patient, histological examination of skin tissues in the affected areas, and immunological studies. Treatment of the disease includes immunosuppressive and cytotoxic therapy using glucocorticosteroid 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. Treatment is long, therapy begins with high dosages of steroids, gradually lowering the dose over 6-9 months. Given that many patients with bullous pemphigoid are elderly, it is not possible to carry out a 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 topical application of ointments based on them.

The therapy of bullous pemphigoid with immunosuppressive agents, for example, cyclosporine, has good results. Similarly, cytostatic agents are used - methotrexate, cyclophosphamide. Double filtration can significantly accelerate recovery and increase the effectiveness of therapy for bullous pemphigoid. Outwardly, 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 in this case, 15-20% of patients then relapse.

Forecast and prevention of bullous pemphigoid

The prognosis of classic bullous pemphigoid is uncertain in most cases. This is due to the fact that the disease is chronic and difficult to predict, and most patients are elderly people, often with other comorbidities. 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. Children and adolescent forms of this pathology in most cases are successfully cured. Persons suffering from bullous pemphigoid or successfully treated, should avoid exposure to the skin of traumatic factors - ultraviolet radiation, high or low temperatures, mechanical trauma. This can provoke the development of a relapse of the disease.

Bullous pemphigoid is a relatively common skin disease, which by its external manifestations resembles a chronic course 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 herpetiform dermatitis. This is chronic, which is accompanied by the appearance of a large blistering rash on the skin (external symptoms sometimes resemble true pemphigus).

It should be noted that the vast majority of patients with this diagnosis are people aged 65 years and older. Naturally, medicine knows exceptions, 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 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 more ailments, the clinical picture of which is quite similar:

• Benign non-acantholytic pemphigus, in which the disease 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 50 years of age, although sometimes the disease is also recorded among male patients.

Causes and pathogenesis of bullous pemphigoid

Unfortunately, the mechanism of the occurrence 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, 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.

There is evidence for this theory. 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 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. However, a factor capable of activating the disease is required. It could be:

• vaccination against certain diseases;
• damage or severe irritation of the skin;
• exposure to ultraviolet radiation (long sunbathing, solarium abuse, etc.);
• thermal burns of the skin;
• 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;
• 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 violations 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 bullous pemphigoid (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 happens less frequently.

The main elements of the rash are vesicles and blisters with tense tires. Inside they contain a liquid, usually transparent, but sometimes you can see blood impurities. Often the skin around the blisters turns red.

The term of "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 on the surface are practically not formed, 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. In elderly patients, whose body is depleted by frequent relapses, there is also a decrease in appetite, weight loss, and progressive weakness.

Histogenesis, histopathology and pathomorphology

The pathomorphology 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 larger structures. Along with this, there is also a sharp swelling of the tissues of the dermis.

The lid of the bladder 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 with an electron microscope, it can be seen that the so-called BPAg1 antigens, which are released during the immune reaction, 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 course 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 degranulation of mast cells is also observed.

Histologically, in the disease, there is a detachment of the epidermis from the dermis, between which a subepidermal blister is formed. 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 in diagnosing a disease such 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 epithelialization of erosion proceeds quickly.

The epidermis peel test was 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 pemphigus vera and herpetiformis.

What treatment is considered effective?

What to do if you have bullous pemphigoid? Treatment in this case requires complex. Moreover, the selection of recreational activities and medicines depends on many factors, including the severity of the disease, the age and general health of the patient, and the presence of comorbidities. 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 given intravenously and the dose is gradually reduced as symptoms disappear.

Cytostatics and immunosuppressants, which help to 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, 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 (for example, Furcocin), 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 health. Take it twice a day, 30 drops.
• For external treatment of the rash, 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 cover the compress with plastic wrap.
• For the same purpose, fresh juice or decoction of nettle leaves can be used. The compress is done according to the above scheme.
• Bullous pemphigoid, more precisely, its symptoms can be alleviated with the help of a special herbal decoction. To prepare it, take an equal amount (50 g each) of eucalyptus leaves, serpentine rhizomes, fruits of Japanese Sophora, 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 during 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 proceeds in most cases not 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 not 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 health.

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.

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 the signs of pemphigus due to the formation of blisters on the epidermis. Let us consider in more detail the features of skin lesions, symptoms, methods of treatment.

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 (bulls) that appear on the surface of the dermis. Lever bullous pemphigoid is more common in people over 65 years of age.

Bullous pemphigoid is characterized by tense blisters that form under the epithelium due to delamination 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;
• arms.

Pemphigoid bullous experts may also call senile herpetiform dermatitis, parapemphigus. Bullous lesion of the dermis is considered chronic, it is characterized by relapses. Unlike pemphigus vulgaris, bullous dermatitis occurs without acantholysis. The occurrence of bubbles inside the epidermis is considered a secondary process. This feature was discovered by Lever in 1953. Very rare cases of manifestation of the pathology of the dermis in children, adolescents (only about a hundred cases).

Scientists have found a link between bullous dermatitis and oncological 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 urinary cancer.

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

Causes

Experts note that Lever's bullous pemphigoid is autoimmune in nature. Usually, its development is provoked by a hereditary predisposition to failures in the autoimmune system. Also, experts admit the possibility of a viral etiology of the considered form of dermatosis. 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, the blood of the patient.

Development of the pathological process

After the impact of any of these factors on the immune system, a humoral cellular response occurs. It is manifested by the production of antibodies to certain cells of the epidermis, which have become “alien”. The autoimmune process is activated, there is a rupture of connections between cells in the lower layer of the skin. This is how vesicles are formed, containing liquid inside.

The formed bubbles merge. Dense tires appear on them, which are represented by healthy cells of the epidermis. The cells of the walls of the formation grow old, 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 bubble 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, there are areas with infiltrate.

The fluid from the bladder contains immune cells, a certain number of eosinophil leukocytes. With the development of any processes in the affected area, the connection between the cells of the spinous layer is preserved (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's pemphigoid.

Characteristic signs of the disease

Usually, before the appearance of bubbles, the patient may show only mild signs of the development of the disease. 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.

Bubbles appear only after a certain period of time. Their size reaches 3 cm. In 30% of bullous pemphigoid, erosions occur in the mucous membranes of the vagina and oral cavity. A feature of the bubbles is also considered the strength of their cover. The bubbles that have arisen during pathology are characterized by injury resistance. Inside the formations contains serous fluid, sometimes it is replaced by hemorrhagic, purulent contents.

The opening of the bladder is accompanied by exposure of erosions on the skin, which are characterized by moisture, tenderness of the surface, and a reddish color. The epidermis in these places heals quite quickly, after the wounds, traces are practically not visible.

Of the additional signs of bullous pemphigoid in patients are manifested:

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

With severe exhaustion of a person suffering from bullous pemphigoid, death is possible. Bullous dermatitis is characterized by a chronic course (signs of pathology gradually subside, then they may reappear). Literally in 15 - 30% of cases, doctors observed spontaneous healing of the body.

Scarring pemphigoid

In medical practice, the term "scarring pemphigoid" arose simultaneously with the term "bullous pemphigoid" due to Lever's allocation of this rare dermatosis to a separate group. This pathology is autoimmune in nature.

A feature of this form of pathology is the occurrence of bullae in one place. Bubbles appear on the dermis for a long time. For this reason, scars form. The localization of the disease on the conjunctiva is considered dangerous because of the likelihood of developing blindness.

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

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

Endogenous factors are:

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

Exogenous factors are:

• epilation of eyelashes;
• strong hyperinsolation;
• bite correction with braces.

With cicatrizing pemphigoid, damage to the mucous membranes of the conjunctiva, nasal cavity, mouth, esophagus, pharynx, and genital organs is observed.

Doctors fix the defeat of the mucous membranes in 70% of cases, the pathology of the skin is observed less often (about 30 - 40% of cases).

The development of pathology in the conjunctiva is manifested by such signs:

• edema;
• hyperemia;
• pain syndrome;
• photophobia;
• rashes (small blisters, the size of which reaches the head of a pin).

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

Diagnostics

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

The specialist will need a complete blood count. This diagnostic method shows the following picture:

• eosinophilia (moderate);
• leukocytosis.

Conducted immunoprecipitation reaction makes it possible to see IgG in the composition of peripheral blood that can bind to antigens.

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

• light microscopy. This diagnosis visualizes the epidermal fissure, the presence of a subepidermal bladder, which gradually turns into an intraepidermal one. The study shows a strong 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 group G immunoglobulins, compliment fractions 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:

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

Basic therapeutic methods

Bullous pemphigoid is treated with medications. The first-line drugs are glucotricosteroids (methylprednisolone, prednisolone). The therapy of the pathology under consideration is quite long, it begins with large doses of steroids. For a course of therapy of 6 to 9 months, the dosage of drugs is gradually reduced.

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

The therapy of bullous dermatitis by means of immunosuppressive agents (cyclosporine and others) will be quite effective. Doctors also prescribe:

• cytotoxic drugs (cyclophosphamide, methotrexate);
• antiseptics. They are necessary for the prevention of secondary infection, complications.

To significantly speed up the therapeutic course, doctors recommend plasmapheresis with double infiltration. The entire therapeutic course sometimes takes up to two years. Even after the end of treatment of bullous pemphigoid, relapses are possible in 15-20% of patients.

Disease prognosis

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

Previously, specialists diagnosed many cases of death in bullous pemphigoid, but doctors did not take into account the age of patients, the severity of comorbidities. Treatment of children and adolescents is carried out successfully.

Those who have been ill with bullous pemphigoid should avoid exposure to the dermis of negative factors:

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

See also other skin diseases