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December 26, 2016 00:02

Bullous pemphigoid |The symptoms and treatment of bullous pemphigoid

bullous Pemphigoid (synonyms: pemphigoid, parapemfigus, senile pemphigus, dermatitis herpetiformis senile) - autoimmune disease developing, usually in persons over 60 years, including as a paraneoplaziya.It can occur in children.Pemphigoid is a benign chronic disease, whose clinical picture is very similar to pemphigus vulgaris, and histological - with dermatitis herpetiformis.

causes and pathogenesis of bullous pemphigoid

In recent years, studies have shown that in the pathogenesis of dermatosis important role played by autoimmune processes.Bullous pemphigoid patients serum and cystic fluid LgG-detected antibodies, lgA-antibody to the basement membrane deposition IgG, rarely - IgA and NW-component of complement in basement membrane such as skin or mucosa.It is found that the titer of antibodies and circulating immune complexes at pemphigoid correlate with disease activity.

Symptoms of bullous pemphigoid

The disease usually occurs in people of both sexes aged over 60, but can occur at any age.The

main clinical sign is the presence of tense blisters appearing on eritemato-edematous background, rarely on unaltered skin and preimushestvenno localized on the abdomen, extremities, in the folds of the skin, in 1/3 of the cases in the oral mucosa.Observed local centers.Symptom Nikolsky negative Ttsanka cells do not show.In some cases, there may be a polymorphism rashes, scarring, mostly in benign mucosal pemphigoid obodochek and local scarring pemphigoid.There are observations about the combination of changes and scarring bullous rash common in children with IgA deposition in dermoepilermalnoy zone against the background of low titers of IgA antibodies against the basement membrane, which is treated as a child scarring pemphigoid linear IgA-deposits, if the combination of the process excluded other pathologies.The disease begins with the appearance of blisters on erythematous or erythematous, urticaria spots, rarely - the seemingly intact skin.Bubbles are usually located symmetrically, rarely celebrated herpetiformis rashes.Bubbles in size from 1 cm to 3 have a round or hemispherical shape, filled with a transparent serous content, which can then be converted into purulent or hemorrhagic.Through thick tires they are very resistant to trauma and clinically similar to dermatitis herpetiformis.Large bubbles are sometimes not so tense and looks very similar to those at pemphigus vulgaris.Along with bubbles appear small and large urticaria rash of red or pink-red color stagnant.This is particularly evident at the time of dissemination of the process, when erythematous phenomenon around the bubbles regress or may completely disappear.After opening the bubbles are formed slightly moist pink-red erosion, which epiteliziruyutsya quickly, sometimes even on the surface do not have time to form crusts.Increased erosion size usually is not observed, but their peripheral sometimes marked growth.The favorite places of localization of bubbles are folds of skin, forearms, inner surface of shoulders, torso, inner thighs.The defeat of mucous membranes is unusual, but the resulting erosion, clinically similar to erosion at ordinary pemphigus on the mucous membrane of the mouth or vagina.

Subjectively rash accompanied by a slight itching, rarely - itching, pain and fever.In severe disseminated within and also with the elderly and malnourished patients the loss of appetite, general weakness, weight loss, and sometimes - fatal.The disease is prolonged, periods of remission interspersed by periods of relapse.

of chronic disease, the prognosis is much more favorable than in pemphigus.

Pathology bullous pemphigoid

In Nachet process between the cytoplasmic processes of basal cells formed numerous vacuoles, which then merge to form larger subepidermal bubbles amid a sharp swelling of the dermis.Tire bladder is unaltered epidermis, whose cells are stretched, but the intercellular bridges are intact.In the future, there is necrosis of the epidermal cells.Pegeneriruyuschy epidermis, advancing to the edges of the bubble, gradually captures his bottom, causing the bubble becomes intraepidermal, sometimes podrogovym.Inflammation in the dermis expressed differently.If blisters developed on intact skin, the perivascular infiltrates are located.If the bubbles are formed on the background of the inflammatory process, infiltrates in the dermis is very massive.The composition of the polymorphic infiltrate, but mostly dominated by lymphocytes and neutrophils with an admixture especially eosinophilic granulocytes, which can be, and in the contents of the bladder among fibrin strands.When immunomorfologicheskie study infiltrates M. S. Nester et al.(1987) found in the lesions large number of T-lymphocytes.including T-helper and T-suppressor, macrophages, and macrophages intraepidermal.Such composition infiltration suggests the role of cellular immune responses in the formation of bubbles with the involvement of macrophages process Electron microscopy of the lesions in different stages of the process has shown that the earliest stages observed swelling of upper parts of the dermis, and between the basal cells within the basal membrane formation zonesmall vacuoles.Later, there is an expansion of the space between plasmolemma basal cells and basal lamina, which is the base of the bladder.Then she partially compacted and destroyed.Scion of basal cells in contact with ANDi cells (filtrate dermis, eosinophilic granulocytes penetrate the epidermis and defanuliruyut it, in 40% of cases there is eosinophilic spongiosis with the presence of cystic fluid chemotactic factor. In 50% of cases in the basement membrane zone revealed globular cells that histologically, ultrastructural and immunologically different from those not in lichen planus, lupus erythematosus, dermatomyositis and other dermatoses. The method of direct immunofluorescence J. Horiguchi et al. (1985) found in their immunoglobulin G and M, C3 complement components, and fibrin. The originthese cells are involved destructively altered epithelial tire bladder.

differentiation of this disease from pemphigus vulgaris is not difficult, even with intraepidermal vesicle localization. For pemphigus characterized by primary changes in the epidermis, which formed acantholytic blisters while at pemphigoid acantholysis missing,and changes in the epidermis are secondary.Distinguish bullous pemphigoid from diseases with subepidermal blistering localization is very difficult, often impossible.Blisters developed on the basis of non-inflammatory, can not contain eosinophilic granulocytes, to the time they are difficult to differentiate from bubbles with epidermolysis bullosa or late cutaneous porphyria.Blisters caused on inflammatory basis, it is very difficult to distinguish from the bubbles in benign mucous membrane pemphigoid and dermatitis herpetiformis.Benign mucous membrane pemphigoid they observed more intense precipitation bubbles than with pemphigoid.Unlike dermatitis herpetiformis bullous pemphigoid with no papillary microabscesses forming further multi-chamber bubbles.From multiforme exudative zritemy bullous pemphigoid by the absence of perivascular eosinophilic granulonitov located near dermal papilla, mononuclear infiltrate nature near dermoepidermalnogo connections and early epilermalnyh changes as spongiosis, exocytosis, and necrobiosis.In all difficult cases neobholimo holding immunofluorescence diagnostics.

Histogenesis bullous pemphigoid

pemphigoid, pemphigus like, refers to an autoimmune dermatoses.The antibodies in this disease are directed against two antigens - BPAg1 and BPAg2.Antigen BPAg1 located at fixing poludesmosom keratinocytes in the basal layer, the antigen BPAg2 is also in the area and allegedly poludesmosom formed collagen type XII.

immunoelectron microscopic study using antiperoksidaznogo-peroxidase method showed IgG localization, C3 and C4 components of complement in basement membrane lamina lucida and lower basal surface zpiteliotsitov.Furthermore, complement component C3-detected on the other side of the basement membrane - in the upper dermis.In some cases, the deposits are IgM.Circulating antibodies to basement membrane zone are found in 70-80% of cases, which is for patogmonichnym pemphigoid.There are a number of works showing the dynamics of immune-morphological changes of the skin in areas of blistering.Thus, I. Carlo et al.(1979).studying the skin near the lesion, discovered beta1-lobulin - plasma protein that regulates the biological activity of the complement component C3 in the basement membrane zone along a NW-component of complement revealed immunoglobulin G. T. Nishikawa et al.(1980) found antibodies against basal cells in the intercellular spaces.

The histogenesis bladder also play the role of enzymes secreted by cells infiltrate.It has been found that macrophages and eosinophils accumulate near the basement membrane, then migrate through it to accumulate lamina lucida and spacing between the basal cells and the basement membrane zone.Furthermore, in response to activation of complement occurs expressed mast cell degranulation.Enzymes secreted by the cells, causing the degradation of tissue and thereby contribute to the formation of the bubble.

Histopathology Histologically, there is detachment of the epidermis from the dermis to form subepidermal bubble.Acantholysis not marked.As a result, early recovery bubble bottom and the peripheral part podepidermalny bubble becomes like intraepidermal.The contents of the bladder consists of histiocytes, lymphocytes with an admixture of eosinophils.

bottom of the bladder is covered by a thick layer of white blood cells and fibrin.The dermis is edematous, diffusely infiltrated and consists of histiocytic cells, lymphocytes, eosinophils varies.

vessels dilated, their swollen endothelium.Due to the lack acantholysis Tzanck cells in the smears are not available.There location IgG and NW component of the complement along the basement membrane.

differential diagnosis

bullous pemphigoid should be distinguished from the true pemphigus, dermatitis herpetiformis Duhring, exudative erythema multiforme and others.

Treatment of bullous pemphigoid

The therapy depends on the severity and prevalence of process.Therapy should be complex and individual.The main therapeutic agent - glucocorticosteroid medications that are prescribed at the rate of 40-80 mg of prednisone daily with a gradual decrease.Perhaps the appointment of higher doses of the drug.The encouraging results observed with immunosuppressants (cyclosporin A) and cytotoxic drugs (methotrexate, azathioprine, cyclophosphamide).There are reports of high therapeutic efficacy of corticosteroids in combination with methotrexate, azathiprine or plasmapheresis.To improve the effectiveness of therapy prescribed in conjunction with corticosteroids systemic enzymes (Phlogenzym, Wobenzym).The dose depends on the severity of the disease and on average 2 tablets 2-3 times daily.Externally applied aniline dyes, creams, ointments containing glucocorticosteroids.