What is True Pemphigus?
True (acantholytic) pemphigus is a chronic relapsing, hormone-dependent disease, during which a generalized spread of blisters occurs, as a result of which the general condition of the body suffers. The disease has a progressive course. Sometimes periods of remission of varying severity and duration can occur. Persons of 40-60 years are usually ill.
Causes of True Pemphigus
The causes and mechanisms of pemphigus development are unknown, although there are a large number of theories. These include: infectious, immunological, metabolic, neurogenic, endocrine, enzymatic, toxic. The most recognized cause by most researchers is the immunopathological auto-aggressive processes in the body. This is confirmed by the detection of antibodies against intercellular substance in the affected skin, vesicle fluid and blood serum of patients.
The use of the immunofluorescence method of examination confirms the presence of a luminescence characteristic of pemphigus patients only in the intercellular space in the spine-shaped epidermis layer. It is most likely that autoimmune mechanisms in pemphigus are of pathogenetic rather than etiological significance.
Pathogenesis during True Pemphigus
Some researchers consider all forms of pemphigus as types of one disease, which is caused by a filtering virus or a group of biologically related viruses. The authors of this theory give their evidence, such as:
- infection of white mice and rabbits with blister contents;
- infection of chicken embryos with material from pemphigus patients with blistering in embryos;
- a positive complement binding reaction prepared from the contents of the blisters and sera of patients with pemphigus and Dühring’s dermatosis;
- detection of virus-like formations in pemphigus cells by electron microscopy, detection of the cytopathogenic effect of blood serum and contents of the blisters of patients with pemphigus and Dühring’s dermatosis using the tissue culture method and others. But all these data are not absolute evidence of the viral nature of the disease.
There is also a neurogenic concept of the appearance of pemphigus, which is one of the first that was supported and developed by P.V. Nikolsky. He considered pemphigus as a neuro-trophic disease, which is caused by the degeneration of nerve cells. In favor of this theory, there are cases of the development of dermatosis after neuropsychic injuries, severe emotional upheavals. In some cases, patients who died from pemphigus showed degenerative changes in the cells of the spinal and medulla oblongata and the spinal ganglia. These changes are most likely pathogenetic in nature.
The exchange theory is based on significant changes in water, mineral, and protein metabolism observed in pemphigus patients. Sodium chloride metabolism is regulated by the function of the adrenal cortex. This function is called mineralocorticoid. This theory has clinical evidence: efficacy in the treatment of pemphigus steroid hormones, increased excretion of glucocorticoids in such patients, which confirms inhibition, up to the depletion of glucocorticoid function of the adrenal cortex in them. For the endocrine concept of etiology of pemphigus, there are known cases of the occurrence of this disease in pregnant women and its disappearance after childbirth. But it is most likely that metabolic and endocrine disorders are not primary in pemphigus, but occur a second time in response to the influence of some other factor. Among other things, there are single observations of hereditary transmission of pemphigus.
Symptoms of True Pemphigus
The existing classification distinguishes 4 forms of true pemphigus:
- vulgar (ordinary);
- vegetative;
- leaf-shaped (exfoliative);
- seborrheic.
True pemphigus often affects women. The preferred age for the occurrence of pemphigus fluctuates between 40 and 60 years. It is rare in childhood and adolescence and is characterized by a severe, often malignant course, up to a fatal outcome.
Vulgar pemphigus
Pemphigus vulgaris is the most common form of all forms of true pemphigus (in approximately 75% of cases). In this type of dermatosis, the mucous membranes of the oral cavity and pharynx are first affected, and then the body, extremities, face, external genitalia, inguinal folds and axillary cavities are involved in the process of the skin. The disease has a severe chronic course. A sudden onset is characteristic when, for no apparent reason, against the background of unchanged skin, initially intense, and then quickly becoming sluggish, blisters of small sizes appear. In the initial stages of the disease, they are filled with a transparent serous fluid, which later becomes cloudy. Weeping erosions of various shapes quickly form under the bubbles. Healing of eroded surfaces ends with the formation of layered crusts of brown color, after which brownish pigmentation remains.
There are cases of spontaneous improvement (without treatment), which are replaced by exacerbations of the disease. If the course of pemphigus is benign, then the general condition of the patient remains practically unchanged. The general serious condition and exhaustion of the body, septic fever, which is especially pronounced when a secondary infection is attached, is observed with a malignant course of the disease. In these cases, eosinophilia is expressed in the blood of patients, the protein content is reduced, protein fractions containing immunoglobulins A, J, M are altered. Lesions of the liver, kidneys, heart and lungs are quite common. With pemphigus, itching almost never occurs. Numerous erosions are painful and intensify with a change in body position, dressings. Lesions cause especially painful pains when they are localized on the mucous membranes of the oral cavity, genitals, etc.
An important clinical diagnostic sign of pemphigus is the symptom, or phenomenon, of Nikolsky. It is caused by degeneration in the malpigium layer of the skin, the so-called “acantholysis,” and consists in the fact that when you pull on a scrap of the bladder covers, the epidermis detaches on apparently healthy skin. Also, with friction of the skin, which seems healthy and is located between the bubbles, there is a slight rejection of the upper layers of the epidermis.
With pemphigus, there is a diagnostic test called the Asbo-Hansen symptom. It consists in the fact that when you press on the bubble that has not yet opened with your finger, you can see how the liquid in the bubble exfoliates adjacent areas of the epidermis and the bubble itself in the eyes increases in size along the periphery. But Nikolsky’s symptom for true pemphigus is not a strictly mandatory sign of only this disease, although it is of great value for diagnosis. This symptom also occurs with other dermatoses: congenital bullous epidermolysis, Ritter’s disease, Lyell’s syndrome. In almost all pemphigus patients, this symptom is positive only in the acute phase. In other periods of the disease, it is negative.
A well-chosen treatment of patients with true pemphigus significantly prolongs the periods of remission (improvement) and delays the onset of relapse, and maintenance therapy using steroid hormones saves lives for many years.
Vegetative pemphigus
At the initial stages of development, this form of pemphigus is clinically similar to vulgar pemphigus and often begins with the appearance of blisters on the mucous membranes of the oral cavity. But with vegetative pemphigus, a tendency of the bubbles to be located around the natural holes, the navel, and also in the area of large folds of the skin (axillary, inguinal-femoral, intergluteal, under the mammary glands, behind the auricles) is observed. When the bubbles open, erosion is formed in their place, covered with a dirty coating and papillomatous growths. In this case, a large amount of exudate is secreted. There is a tendency to merge lesions and the formation of extensive vegetative surfaces, in some places with purulent-necrotic decay. Nikolsky’s symptom is often positive. Patients feel pain and burning, which impede active movement.
With successful treatment, vegetation is compacted, dried, epithelialization of erosion occurs with pronounced residual pigmentation. But some patients may quickly develop exhaustion, and some of them die.
Leaf-shaped (exfoliative) pemphigus
For this variety of pemphigus, sharp acantholysis is characteristic, which leads to the formation of surface cracks, which later turn into bubbles. In adults, leaf pemphigus is more common than vegetative, and in children it prevails over other types of pemphigus vulgaris.
The disease begins on the apparently unchanged skin with the appearance of flabby, with a thin cover of blisters that protrude slightly above the surface. The opening of the bubbles is quite fast, and in their place extensive erosion is formed. But most often, the bladder coats dry out, forming thin lamellar scaly crusts. Under crusts, erosion epithelizes slowly. Layered crusts are formed due to the formation of new portions of exudate (detachable) and resemble puff pastry, therefore this type of pemphigus is called “leaf-shaped” – exfoliative.
The symptom of P.V. Nikolsky, described in 1896, namely with pemphigus pemphigus is always sharply positive. The disease has a long-term chronic course with periods of spontaneous improvement. As a result of progression, the process gradually occupies large areas of the skin, up to the scalp, and often proceeds as an erythroderma. Very rarely affected are the mucous membranes of the oral cavity. The severity of the general condition of the patient depends on the extent of skin lesions. If the foci are small, then the condition is disturbed little, and with generalized lesions there is an increase in temperature, disturbances in water and salt metabolism, the progression of anemia, eosinophilia. Patients in such severe cases weaken, lose weight, up to the development of cachexia.
Seborrheic (erythematous) pemphigus – Senir-Asher syndrome
This disease is a true pemphigus, as it can turn into a leaf-like or vulgar form.
The course of seborrheic pemphigus is long, but in most cases benign. Most often, the skin of the face, chest and back is first affected. Sometimes the process affects the scalp. Close to each other, fatty crusts form on reddened skin, i.e., on an erythematous background. The process on the face is similar in appearance to the picture of scarring erythematosis.
Wet erosion is found under the crusts. If a smear imprint is made from these eroded surfaces, then anatomical cells can be detected in it. Quite often, the bubbles are invisible, and as a result of this, a false idea of the primacy of the crusts is created. Seborrheic pemphigus rarely appears on the mucous membranes, but if a pathological process develops on them, then this is a poor prognostic sign.
This type of pemphigus is characterized by intraepidermal abscesses (intradermal abscesses), which are composed of eosinophils (leukocytes).
Diagnosis of True Pemphigus
If the disease proceeds classically, then the diagnosis is not difficult. It is necessary to differentiate pemphigus from multiform exudative erythema, bullous toxicoderma, herpetiform dermatosis Dühring, congenital bullous epidermolysis, bullous form of lupus erythematosus.
True Pemphigus Treatment
Steroid hormones are prescribed according to the scheme. First, treatment is carried out in shock doses. Prednisolone or methylprednisolone – 40-60 mg, triampcinolone – 32-48 mg, dexamethasone – 4-6 mg per day. In the case of the cessation of the appearance of new rashes, a decrease in the phenomena of exudation in the lesion foci, the dose of hormones is reduced, but they are done very slowly to avoid exacerbation of the process and in order to prevent the “phenomenon of withdrawal” of steroid hormones.
A maintenance dose of hormones is prescribed strictly individually for a particular patient, so that it is minimal and does not lead to a relapse of the disease. Such treatment is carried out for a long time. To reduce the dose of corticosteroid hormones and avoid the serious complications caused by them, hormones are combined with the simultaneous administration of cytostatics – immunosuppressants, which include: methotrexate, prospidine, azathioprine. Anabolic hormones are also used – methylandrostinolone, nerobolyl, methylandrostenediol, calcium, potassium preparations, agents for increasing phagocytosis (pentoxyl, methyluracil, ascorbic acid, vitamins, rutin, ascorutin, riboflavin, calcium pantothenate, folic acid). In case of attachment of a secondary infection, antibiotics are used.
Symptomatic therapy is of great importance, which depends on the general condition of the patient – this is a blood transfusion, native plasma infusion, the introduction of y-globulin, liver preparations (heparin, vitagepancreatinum, Hepavit tablets), autohemotherapy.
Patients must follow a balanced diet of protein, calcium and vitamins. If the patient has large lesions of the skin and mucous membranes, frequent warm baths are prescribed with disinfecting solutions, for example, potassium permanganate, followed by the use of disinfectants: Castellani liquid, 1-2% aqueous solution of pioctanin, 5% boron naphthalene ointment, etc. Affected areas of the skin are lubricated with corticosteroid ointments (Lokacorten, Flucinar, Dermazolon, Oxycort, Gyoksizon, etc.).