Pemphigus disease: symptoms, photo, treatment in children and adults

Pemphigus - chronic autoimmune disease characterized by the appearance of special form bubbles on the surface of the previously healthy skin and mucosa. Among the types of pemphigus can be distinguished: the vulgar, vegetative, erythematous, and the sheet.

Pemphigus can be diagnosed in the case of detecting acantholytic cells that identify in smears or bubbles in the composition in the epidermis (histologically). For treatment of pemphigus first of all applied glucocorticosteroids (designate a reception rate). The latter always goes well with extracorporeal blood correction (plasmapheresis, krioaferoz, hemosorbtion).

What it is?

Pemphigus - a serious disease that affects the human skin. As a result of its progression to skin and mucous membranes are formed pathological bubbles, inside filled with exudate. This process begins because of epithelial stratification. Pathological lesions may coalesce and have a tendency to rapid growth.

Causes of

The causes of pemphigus have not yet fully understood. One of the main causes of pemphigus is a violation of the autoimmune process, thus the immune system cells are antibodies.

Violation cell structure falls under the influence of external factors, as well as corrosive environments. As a result, communication between cells is disrupted, resulting in the formation of bubbles. The percentage of morbidity in people with a hereditary predisposition is much higher.

Bubble formation mechanism

Human skin can be figuratively described as the water-spring "mattress", covered with a kind of "wall". In the formation of bubbles "mattress" is not involved - suffers only the top layer, the epidermis.

Epidermal layer consists of 10-20 cell layers that appear under the microscope as the building blocks. "Building Blocks" of the second layer of the epidermis are connected with each other sort of "bridges". On top of the "wall" are layers of cells, not quite like cell-like application of the cream. It - scales, corneocytes are necessary for protection against mechanical, chemical and physical damage.

If under the influence of internal or external causes formed antibodies, which are destroyed by "Bridges" between -desmosomy basal layer of cells (called acantholysis and seen under the microscope), it is - true pemphigus. If tissue fluid penetrates between the basal and upper layers of the epidermis without destroying the "bridges" is - pemphigoid. Without degradation of desmosomes and flows viral pemphigus.

Classification

Varieties neakantoliticheskoy pemphigus:

1. Neakantoliticheskaya pemphigus benign nature. Pathological elements are formed exclusively in the human oral cavity. On examination can reveal inflammation, as well as its minor pitting.
2. Bullous pemphigus neakantoliticheskoy form. It is a benign disease that develops in adults and children. Formed on the skin blisters, but no signs of acantholysis. These pathological elements may disappear spontaneously without scarring.
3. Scars neakantoliticheskaya pemphigus. This is called pemphigoid pemphigus in the medical literature eyes. It is most commonly diagnosed in women who have crossed 45 years of age limit. The characteristic symptom - the defeat of the visual apparatus, skin and oral mucosa.

Classification of true pemphigus:

1. Erythematous form. This pathological process combines several diseases. Its symptoms are similar to seborrheic dermatitis, erythematous variant of systemic lupus, as well as the true pemphigus. Pemphigus erythematosus in adults and children is very hard treatable. It is worth noting that the disease is diagnosed, not only in humans but also in some animals. The characteristic symptom - in the cutaneous manifestation of the body cover and face red spots, top crusty. At the same time a sign on the scalp seborrheic manifestations occur.
2. Pemphigus vulgaris. This type of pathology is diagnosed in patients more frequently. On the skin, forming bubbles, but the signs of inflammation are observed. If you do not spend time treatment of pemphigus, the pathological elements are spread throughout the skin. It is worth noting that they can merge and form larger lesions.
3. Foliaceous pemphigus. The name of this form was due to the nature of the pathological elements. On the skin of the human form bubbles, which hardly rise above the epidermis (not stretched). They are formed on top of the crust, which have the property of layered on top of each other. Creates the effect of sheet material folded into stacks.
4. Brazilian pemphigus. It has no restrictions on age and gender. Cases were reported of its development and in young children, and elderly people aged 70 to 80 years. It is also possible its progression in people of middle age. It is worth noting that this species is endemic, so only found in Brazil.

symptoms

Given that experts have identified several different types of this disease, the symptoms, and each of them will be very specific. Of course, there are a number of general trends and features inherent in all types of the disease. These include, for example, undulating course of the pathological process.

periods of exacerbation alternating with the transition of pemphigus in a more relaxed stage when the main symptoms subside or disappear altogether. An important factor for the patient would be the fact that in the absence of timely diagnosis and destination effective course of treatment, a high risk of severe conditions, burdened with attendant diseases.

• The presence of scabs, from pale pink to red soft and dense, resembling shingles;
• It occurs general deterioration;
• Reduced immune response;
• Bubble formation of different density;
• Also it marked in severe separation of layers of the epidermis, where it can occur both in the lesion, and away from it.
• Damage and ulcers of the oral mucosa, nasal or genitalia;
• Pain during an act of swallowing or when eating;
• Halitosis, indicating the lesion mucous membranes;
• Hypersalivation or, in other words, increased salivation;
• In seborrheic form on hairy scalp exhibit characteristic yellowish brown or brown-brown.
• Bubbles different appearance from planar and ending with thin-walled, which burst with a slight touch. In their place are formed erosion and, further, peel.
• When severe, the formation of eroded surface of the skin at the site of bubbles. They feature becomes a tendency to peripheral growth. Over time, such erosion occupy most of the surface of the skin, causing the patient pain and discomfort.
• In children, symptoms of pemphigus localized over the entire surface of the skin, including the extremities.

Experts say that in this disease can be observed as the pure form of the pathological process, and mixed, passing smoothly into one another. Consequently, the signs and symptoms of pemphigus in a particular person may vary and indicate the presence of several types of diseases.

What is pemphigus: photo

The photo below shows how the disease manifests itself in humans.

Diagnostics

Experts say that to put the correct diagnosis can be based on a comprehensive examination of the patient, which includes several important steps:

1. Examination of the patient for the presence of the clinical picture. At this point, the doctor prescribe the nature of lesions, their localization, the degree of disease, etc.
2. Cytological analysis required to determine the presence of cells in smears akantolicheskih biomaterial.
3. Carrying Nikolsky samples, allowing to differentiate pemphigus from similar to her pathological processes.
4. Method of direct immunofluorescence. This study reveals the presence of immunoglobulin in the intercellular substance of the epidermis.
5. Histological examination, which is based on the methods of detecting cracks and other damage within the epidermis.

Only the combination of all the results allows an accurate diagnosis and effective treatment, leading to the recovery of the patient.

Treatment of viral pemphigus

Treatment of viral pemphigus involves the appointment of the following systemic medications:

• cytostatics stop dividing immune cells: Sandimmun, azathioprine, methotrexate;
• antivirals: Viferon, Laferon TSikloferon;
• glucocorticoids: dexamethasone, prednisolone;
• antipyretics: Ibuprofen, paracetamol, Nimesil, mefenamic acid;
• antihistamines relieve itching: Tsetrin, Diazolin, Fenistil.

For external machining of the affected areas of the skin may designate:

• antimicrobially-local anesthetics for irrigating the mouth, if struck viral pemphigus mucus child: Forteza, Orasept;
• antiseptics: Chlorhexidine, Methylene blue, Miramistin;
• combined preparations of antiseptics and anesthetics: Oflokain, pharmacy mash;
• antipruritic lotions of juice of nettle, aloe, walnut oil.

Since usually children diagnosed treated under stationary conditions, to enhance the therapeutic course of treatment may be carried out procedures for blood purification of antibody:

• Plasmapheresis - replacement of the liquid portion of blood similar solution without bacteria, immune complexes and antibodies;
• hemosorbtion using a charcoal filter.

Only a doctor can say than to treat viral pemphigus, because in each case it can acquire any special features. As for other forms of pemphigus, the therapeutic course for them, too, it is determined individually.

How to treat other forms of pemphigus?

Pemphigus treatment process is quite complex. Therefore, this type of self-treatment of the disease is by no means unacceptable. The disease progresses rapidly, affecting large areas of the skin that leads to disturbances of the internal organs.

Treatment of pemphigus necessarily carried out in the dermatological hospital. First appointed corticosteroids, cytotoxic drugs and other medicines intended for relief of the disease and the duration of life of patients.

Drugs must first be taken in large doses. At the same time pay attention to blood sugar and urine, to monitor blood pressure and observe good personal hygiene. When frequent changes of bedding, underwear to prevent the occurrence of secondary infection.

Preparations for the treatment of pemphigus

The patient is shown receiving glucocorticoid in high doses. To this end, the following drugs can be used:

• metipred;
• prednisolone;
• dexamethasone;
• Polkortolon.

At the beginning of regression of symptoms the dose of these preparations is gradually reduced to the minimum effective. Patients with abnormalities of the gastrointestinal tract prescribe long-acting corticosteroids:

• Metipred depot;
• diprospan;
• Depo-Medrol.

hormonal agents treatment can cause a number of complications, but they are not the reason for steroid withdrawal. This is because of the failure of reception can lead to relapse and progression of pemphigus.

Possible complications in the treatment of:

• acute psychosis;
• arterial hypertension;
• depression;
• insomnia;
• hyperexcitability of the nervous system;
• steroid diabetes;
• thrombosis;
• obesity;
• angiopathy;
• erosion or ulceration of the stomach and / or intestines.

With a sharp deterioration in the patient's condition in patients receiving corticosteroids may be recommended the following measures:

• dieting: restriction of fats, carbohydrates and salt, the introduction of the diet of large amounts of protein and vitamins;
• preparations for the protection of the gastric mucosa: Almagel and others.

Parallel to glucocorticoid therapy to increase the efficiency and reduce the possibility of hormonal agents and dosages are assigned cytostatics immunosuppressants.

This may apply such drugs:

• Sandimmun;
• methotrexate;
• Azathioprine.

For the prevention of disorders of electrolyte balance patient should calcium and potassium. And when secondary infection erosions - antibiotics or antifungal agents.

The ultimate goal of medical therapy is aimed at the disappearance of lesions.

preventive measures

Specific measures to help prevent the development of disease, does not exist. The higher level of immune protection, the less chance for the occurrence of dermatological diseases.

Important:

• control the nature of chronic diseases;
• strengthen the immune system;
• personal hygiene;
• eat right.

Preventive measures pemphigus newborns:

• often change clothes;
• denied care for newborns people with pustular skin lesions;
• regular care of your child's skin;
• strengthens the immune system weakened children;
• need daily wet cleaning, airing of the room.

If you find any rashes on the skin, formation of pustules and blisters immediately consult a dermatologist.

Forecast

The prognosis of acantholytic pemphigus conventionally unfavorable. On the one hand, in the absence of effective treatment of high probability of complications and death.

On the other hand, patients are forced to take pemphigus glyukortikosteroidy long, and sometimes for life, which is fraught with the development of side effects. But the hasty rejection of drugs leads to an immediate relapse of disease. Glyukortikosteroidy do not eliminate the cause of the disease, but inhibit the pathological process and prevent its progression.