What is pemphigus?

It is a serious skin disease that mostly affects the skin and mouth but can sometimes also affect the inside of the nose, the throat, the eyes and the genitals; it starts as burn-like, fluid-filled blisters which soon burst open and turn into superficial sores that crust over. The disease has no other name known in Turkish or in everyday language.

Why and how does pemphigus occur?

In medical terms it is classified as an autoimmune disease. In other words, it is one of the diseases that arise when the immune system – which under normal conditions is a protective system only against microbes and foreign substances that may be harmful to the human body – perceives the person's own tissues and cells as foreign and fights against them. To put it more simply, pemphigus, which we cannot predict who will develop, results from the body perceiving its own skin as foreign for a reason we do not yet know, and reacting against it. As a result of this reaction, the bonds holding together the cells in the uppermost layer of the skin are broken by the effect of substances secreted by the body called autoantibodies. As a result, the cells separate from one another and splits form within the skin. When these split areas fill with serum, blisters filled with clear fluid, called bullae, form. Because the walls of these blisters are very thin, they burst open easily and usually turn into painful, raw-looking, superficial sores. The loss of fluid and certain essential substances from the body through these sores, or infections caused by microbes entering through them, form the basis of the adverse effects of pemphigus, which can sometimes be life-threatening.

Is it hereditary?

Although the disease can rarely be seen in more than one person in the same family, it is not hereditary. In other words, pemphigus in a mother or father is not passed directly to their children.

Is it contagious?

The disease is not contagious. In other words, there is no question of it being transmitted in any way to the patient's spouse, children or those in close contact with them.

How common is the disease?

Pemphigus is a rare disease. Although there are no precise data for our country, it is estimated that roughly 2–3 in 1 million people develop the disease. It occurs equally in men and women. Although it most commonly appears in middle-aged people, it can be seen at any age.

What factors cause the disease or make it more likely to occur?

Although some drugs and chemicals have been shown to cause pemphigus in some people, the exact triggers are unknown. Some pemphigus patients report that their complaints increase after eating certain foods (for example onions, garlic and leeks).

Are there different types of pemphigus?

Although there are many clinical types of pemphigus, the most common is the type we can call deep pemphigus (pemphigus vulgaris), in which the weeping sores on the skin are usually accompanied by mouth sores. The other is superficial pemphigus (pemphigus foliaceus), a milder type with only very superficial sores, crusting and scaling on the skin.

What are the symptoms of pemphigus?

As mentioned above, in the deep type, which is the type of pemphigus we encounter most often, the disease usually begins with mouth sores that do not heal for a long time. After this period of mouth sores lasting weeks or even months, which do not heal with medication in the form of mouthwashes, the disease spreads to the skin as weeping sores, especially on the scalp, face and trunk. These sores first take the form of skin blisters that we call bullae, looking like flaccid, fluid-filled bubbles, and resemble the blistering of the skin after a burn. While their contents initially look like clear water, over time they take on a purulent, yellowish-white appearance. The bullae soon burst open and turn into superficial, weeping sores with a moist base. Over time they dry and crust over. However, if not treated, the disease continues with fresh weeping sores appearing in other places. If these sores appear in the eyes (one or both), they show themselves as burning, redness and watering. If they appear in the nose, they cause bleeding, discharge and congestion. When they appear in the mouth and throat, they cause quite distressing pain that increases especially when eating and swallowing, sometimes stopping the person from eating and drinking. Sometimes shortness of breath and hoarseness may be observed as a result of these sores appearing in the windpipe and vocal cords. These sores can sometimes appear around, on or inside the genitals, and sometimes around the anus.

Where and to which specialist should one go when pemphigus is suspected?

Pemphigus is a skin disease. Therefore, a patient with the above symptoms should first see a dermatologist or a healthcare institution with a dermatologist.

How is pemphigus diagnosed?

A doctor who suspects pemphigus from the symptoms mentioned takes a small piece of skin, usually no larger than a chickpea, from the edge of one of the sores on the skin or in the mouth, numbing only the sore area (local anaesthesia), with a very small operation requiring a few stitches (biopsy), in order to confirm the diagnosis. The diagnosis is confirmed by examining this piece under the microscope (pathological examination) and with a special method called immunofluorescence.

What should a patient diagnosed with pemphigus pay attention to? The importance of adherence to treatment and follow-up.

A person diagnosed with pemphigus should not immediately become deeply worried. With good treatment and regular follow-up, stopping the progression of the disease and rendering it inactive, or even curing it completely, is by no means a remote possibility with today's modern treatment methods. However, it should not be forgotten that pemphigus is a disease that requires a long and demanding treatment process and that it can lead to serious consequences if not treated properly. For a pemphigus patient, following the doctor's treatment recommendations to the letter and not missing check-ups – in short, "good treatment adherence" – is extremely important.

Are there other patient-related factors that affect the treatment and course of the disease?

Yes, many other patient-related factors that accompany pemphigus and already existed before the patient developed pemphigus, such as diabetes, high blood pressure, previous tuberculosis, osteoporosis, cataracts, or previous brain or gastrointestinal bleeding, can directly affect the course and treatment of pemphigus. This is because these diseases can adversely affect pemphigus and its treatment, and the treatments given for pemphigus can also adversely affect these diseases. For this reason, the patient must tell their doctor about them during the examination. The doctor will plan treatment and follow-up taking these conditions into account.

How is pemphigus treated?

The basic aim and goal of pemphigus treatment is to suppress, reduce or completely stop the production of the substances produced by the body called "antibodies", mentioned above, which break the connections between skin cells, causing splits and therefore pemphigus. For this purpose, drugs that suppress the immune system directed against the body itself, or certain methods aimed at clearing the blood of antibodies, are used. The most effective and therefore most frequently used drug for this purpose is "cortisone" (for example, the drug called Prednol is one of the cortisone drugs most commonly used in the treatment of pemphigus). Cortisone, mostly taken by mouth as tablets, forms the cornerstone of treatment because of its "life-saving" effect in pemphigus. Other drugs and methods are generally used to support cortisone treatment or, rarely, as secondary options in cases that do not respond to cortisone treatment. Ultimately, pemphigus treatment in general is based on a delicate balance between the healing effect of cortisone and its side effects.

What Is Pemphigus Vulgaris?

It is a rare autoimmune disease that causes severe blisters on the skin and on mucous membranes such as the mouth, nose and genital area. The blisters have a thin covering and open easily, turning into painful sores (erosions). It does not heal on its own and always requires specific treatment.

What Are the Causes of Pemphigus Vulgaris?

We all have an immune system. It produces the antibodies needed to fight microbial diseases (infections). While these antibodies normally do not harm our own body, in autoimmune diseases they fight against our own body. In pemphigus vulgaris, because of an error in the immune system, the cells of the skin and mucous membranes are perceived as foreign and are damaged by autoantibodies produced against them. The target of these autoantibodies is the structures in the cells of the upper layer of the skin (epidermis) that hold the cells together. Because of the autoantibodies that bind to this desmoglein, the cells in the skin and mucous membranes cannot stay together for long and separate. This is the cause of the blisters and sores that are the typical lesions of pemphigus vulgaris. The disease affects both sexes equally. Although it can start at any age, it is seen in adults. It affects all races, but is often seen in middle age and more frequently in certain groups, for example Jewish people. Pemphigus vulgaris is not a microbial (infectious) disease.

Does pemphigus vulgaris run in families?

The disease is not passed from generation to generation. Genetic factors are important, but other factors are also needed for the disease to become active.

What are the symptoms of pemphigus vulgaris?

Painful sores that do not heal, sleep disturbance, and weight loss due to being unable to eat and drink because of sores in the mouth are seen.

What does pemphigus vulgaris generally look like?

In most patients, blisters and sores first start in the mouth and are later seen on the skin. Rarely, the skin is affected first. On the skin:

• It starts as thin-walled blisters on normal-looking skin (collections of clear

fluid in the skin) and they open easily, becoming sores.

In sores (erosions) on the skin or mucous membranes, the outer layer of the skin is lost.

There is a red, moist, burn-like appearance and a burning sensation.

• The sores crust over. There may be discolouration when they heal.

In the mouth: usually no intact blisters remain in the mouth, because they open easily and sores

(erosions) form. There may be one or two sores or more, and they merge.

What is the course of pemphigus vulgaris?

It does not heal without treatment. Sometimes it flares up, sometimes it follows a better course. There is no way of knowing when it will flare up or get worse. Although it can be controlled with treatment for a long time, it may eventually heal completely, so that no flare-up is seen when treatment is stopped.

How is pemphigus vulgaris diagnosed?

• Because it is a rare disease, most general practitioners never encounter it. When your doctor sees you, they will refer you to a dermatologist.

• A biopsy will then be taken. Separated cells in skin taken from an intact blister are important.

Pemphigus Vegetans

The initial symptoms are the same as pemphigus vulgaris. Bullae form in the mouth and on the skin and, in addition, vegetating masses develop at the sites of the bullae in the armpits and groin. Although the prognosis is poor, it is slightly better than pemphigus vulgaris.
Pemphigus Foliaceus
This is the generalised pemphigus type of the subcorneal group. In these, the roof consists only of the stratum corneum. As a result, the bulla is very fragile. The bullae either burst easily or are resorbed and dry out. When dry, the roof of the bulla looks like scale. There is erythema underneath. At this stage, the disease gives the impression of an erythematous and scaly disease rather than a bullous one, and follows the course of erythroderma. In practice, there is no involvement inside the mouth.
Pemphigus Erythematosus (Seborrhoeicus)
This is the localised pemphigus type of the subcorneal group. It is located on the face, the middle of the chest and the back, which are called seborrhoeic areas. The lesions resemble those of seborrhoeic dermatitis. The scales are greasier in seborrhoeic dermatitis, whereas in pemphigus they are whiter.