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Pemphigus

The pemphigus is a bullous dermatitis, ie a skin disease manifested by the appearance of blisters on the skin and mucous membranes. It is a disease of autoimmune origin, in which the antibodies produced by the body itself destroy skin cells.

To understand this pathology, we will give some basic notions about the structure of the skin. This organ is an external covering of the body, which protects it while communicating it with the environment. It is made up of three layers: epidermis, dermis, and subcutaneous tissue. The epidermis is made up of different cells, of which the most numerous are keratinocytes, which are distributed in several layers and are linked together by intercellular bridges. The dermis is the supporting structure of the skin. It is mainly made up of fibers and contains vessels, nerves and skin attachments (hair follicles, sebaceous glands). Finally, the subcutaneous tissue is made up mainly of fat.

Causes of pemphigus

Pemphigus is an autoimmune disease, that is, it is produced by the synthesis of antibodies by white blood cells, directed against structures of the body itself. In the case of pemphigus, these antibodies are directed against the surface of keratinocytes. In addition, there is a certain genetic predisposition to suffer from it.

The antibodies that are deposited on the keratinocytes favor the destruction of the union bridges between them, appearing accumulation of fluid between them. Subsequently, the fluid-filled spaces become larger, leading to the formation of blisters.

The blisters tend to break early, especially in pemphigus foliaceus; they dry out and give rise to erosions and scabs. They can even cause ulcers, although this only happens when the blisters have a purulent content.

Symptoms of pemphigus

The disease can manifest itself in various clinical forms, however, the basic lesion is the blister, and the characteristic sign is known as Nikolsky’s sign, according to which, by exerting slight pressure on the epidermis, the superficial layer is lifted, exposing a wet surface.

The most frequent clinical forms in our environment are pemphigus vulgaris, pemphigus vegetant, and pemphigus foliaceus.

Pemphigus vulgaris

The blister is the only lesion of pemphigus vulgaris, sometimes being the size of a lentil, and other times the size of a walnut, being flaccid or smooth, with transparent or purulent content. The blisters appear spontaneously or under pressure, and subsequently spread progressively. In a short time they break, and are replaced by erosions, scabs or ulcerations. The most common locations are areas exposed to pressure and friction, such as the back, buttocks, and feet. The appearance of lesions on the oral mucosa is characteristic, spreading to the lips or the skin that surrounds them, although it is difficult to find these lesions in the form of blisters, since they break easily.

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Vegetating pemphigus

In this case, the initial lesion may be a flaccid blister or a pustule. Later it evolves into vegetations, which are wart-like lesions, but which exude pus and appears mainly in the folds (armpits, groin, interdigital areas).

Foliar pemphigus

In this, the bullous lesions last a very short time and, therefore, what we observe are erosive and scaly surfaces. Later, a gradual spread of the lesion appears, affecting the whole body if treatment is not carried out.

How is it diagnosed?

The orientation of the diagnosis is made by the symptoms and the existence of a positive Nikolsky’s sign during the examination, as well as by the cytological study and by the electron microscope; although the definitive diagnosis can only be established by biopsy or by immunological study.

In the cytological study we can verify the characteristics of the keratocytes that appear in pemphigus, which differ from those of normal skin in that they appear separate, as a consequence of the loss of cohesion, and have a rounded rather than cubic shape.

Through the electron microscope we can verify the destruction of the intercellular bridges.

The biopsy shows the presence of acantholysis or separation of epidermal cells, as a consequence of the destruction of intercellular bridges.

During the immunological study we can observe circulating antibodies in the blood or deposited in the intercellular spaces.

Pemphigus treatment

Treatment is based on the administration of corticosteroids, although other drugs can be used in a complementary way, such as immunosuppressants, sulfone, gold, or plasmapheresis.

There is no optimal guideline for the use of corticosteroids, and individualized treatment must be carried out. However, treatment is usually carried out in three phases:

  • A first phase to control the disease, in which high doses of prednisone are used, until the lesions stabilize and stop worsening.
  • A second phase in which the dose is sustained, until the lesions decrease by 80%.
  • Finally, a third phase is carried out in which the dose is reduced, until reaching the minimum dose that is effective, which will be maintained indefinitely in most cases, since only in a third of patients it is possible to suspend treatment completely without symptoms reappearing.

How can I avoid it?

There is no way to prevent it, since it is not an acquired disease, although recurrences can be avoided by maintaining a low dose of corticosteroids.

The existence of pemphigus should be suspected before the appearance of blisters without a justifying cause, and go to the doctor as soon as possible, since it is considered that the sooner the treatment is started, the better the evolution of the lesions.

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Hello Readers, I am Nikki Bella a Psychology student. I have always been concerned about human behavior and the mental processes that lead us to act and think the way we do. My collaboration as an editor in the psychology area of ​​Well Being Pole has allowed me to investigate further and expand my knowledge in the field of mental health; I have also acquired great knowledge about physical health and well-being, two fundamental bases that are directly related and are part of all mental health.

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