Pemphigus: What is pemphigus, why does it occur and what are its symptoms?

Pemphigus belongs to the group of chronic blistering skin diseases (bullous dermatoses).

Pemphigus affects the skin and mucous membranes. Its cause is unknown in most cases.

The following groups of blistering skin diseases are distinguished according to the location and course of blister formation:

1. pemphigus group
2. the pemphigoid group
3. epidermolysis bullosa hereditaria group

Pemphigus-type diseases include life-threatening skin diseases. Severe forms of pemphigus that are not treated can be fatal. Pemphigus is a very rare disease.

The primary symptom of pemphigus is thought to be blisters. Blisters form when the adhesion between adjacent cells loosens. Subsequently, fluid accumulates in the loosened area.

Autoantibodies are directed against the adhesion molecules of the epidermis. The target antigens lose their adhesive function. This results in blistering.

The pemphigus group includes:

• pemphigus vulgaris
• pemphigus vegetans
• pemphigus herpetiformis
• pemphigus foliaceus
• pemphigus erythematosus
• paraneoplastic pemphigus

It is believed that the cause of pemphigus may be a virus or toxic and metabolic disorders.

The whole process of this disease seems to start at the point where the intercellular bridges between skin cells are disrupted or completely missing.

Autoantibodies with a specific affinity for the intercellular components of the epidermis have been demonstrated in patients with pemphigus. These autoantibodies disrupt the strength of the intercellular junctions and thus cause blistering.

Risk factors include...

Geographical origin

Pemphigus is distributed worldwide. The highest incidence of pemphigus is reported in the Near and Middle East, Western Europe and North America.

Interestingly, pemphigus foliaceus is found mainly in rural areas of Brazil and Tunisia.

Ethnic groups

Pemphigus can occur in all ethnic groups. However, patients of Jewish origin are more prone to develop the disease.

Gender and age

In general, pemphigus occurs in both sexes. In some forms, it affects women more often. Pemphigus usually appears between the ages of 30 and 60. Occasionally, it may also appear in childhood or old age.

Genetic predisposition

The higher likelihood of pemphigus in some groups of people may be partly due to genetic predisposition.

Various risk factors (drugs, UV radiation, diet, viruses) can trigger pemphigus. However, these factors only trigger pemphigus in people who have a genetic predisposition for the disease.

Medications

Pemphigus can be triggered by medications that contain the drugs ibuprofen, indomethacin, phenylbutazone, propranolol.

Did you know that...

The results of some studies suggest that in some cases smoking may have a protective effect against pemphigus.

The main symptom of pemphigus is the formation of blisters. They may appear on the skin or mucous membranes (mouth, nose, throat, genitals). Symptoms of the disease may vary in different forms of pemphigus.

1. Pemphigus vulgaris

Pemphigus vulgaris is not a common disease. It is the most common of the pemphigus group (80%).

It occurs between the ages of 30 and 60. In some cases, it can also occur in children.

It is most common in Jews and people living around the Mediterranean.

Pemphigus vulgaris starts subtly. Symptoms can appear anywhere on the skin. Small blisters appear first, which burst after a short time.

In about 50 % of patients, the first symptoms appear in the oral cavity. Symptoms in this area include aphthous stomatitis.

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The manifestations of the disease do not itch in most cases. Typical symptoms of the disease include painful manifestations, fissures, bleeding, crusting and the occurrence of secondary infections.

Pemphigus is characterised by enlarging blisters which gradually spread into the surrounding area. Pressure and pulling of the finger on normal skin causes the surface of the skin to detach and shift.

For the reasons mentioned above, blisters form in areas subject to pressure and friction (back, knees, elbows).

General symptoms may accompany persistent or extensive pemphigus. General symptoms include lack of appetite and progressive wasting. Elevated temperature may be septic in the final stages.

See also:

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2. Pemphigus vegetans

One form of pemphigus vulgaris is pemphigus vegetans. It occurs in approximately one-third of cases of pemphigus vulgaris. The highest incidence of the disease is between the ages of 50 and 70.

There are 2 types:

• Neumann type

Neumann's type develops in the course of pemphigus vulgaris. The main manifestation of the disease is flaccid blisters that burst rapidly. They occur mainly on the lips, in the nasolabial grooves or on the vulva.

• Hallopeau type

Symptoms of the disease appear mainly in the intertriginous areas (axillae, anal region). The primary manifestation is a yellow-coloured blister. After its rupture, there is swelling and the formation of an unpleasant odour.

Such swollen manifestations of the disease cause pain. The problem is the emergence of secondary bacterial or mycotic infection.

Symptoms appear mainly in the following areas:

• perigenital and perianal
• axillary
• submammary
• in the corners of the mouth
• oral and nasal cavities
• in the larynx and pharynx

3. Pemphigus herpetiformis

Symptoms of the disease include blisters of varying size. Patients experience severe itching and burning. The blisters form over the elbows, knees, shoulders and sacral region.

4. Pemphigus foliaceus

This type of pemphigus is very rare.

It affects mainly adults between 50 and 60 years of age. It is chronic in adult patients. Occasionally, it can occur in children. Interestingly, it can heal spontaneously in children.

The disease can be triggered by sunlight or medication. Symptoms of the disease appear mainly in seborrhoeic parts of the body (scalp, face, chest, back). Symptoms of this form of pemphigus never appear on mucous membranes.

This type of pemphigus is characterised by the formation of flat, flaccid vesicles that burst. At the same time, crusts in the shape of leaves and layered scales form. Later, swollen, sticky and moist manifestations are formed, which are characterised by an unpleasant odour.

Fever, secondary infections, diarrhea, decreased urine output and renal failure may occur during the course of the disease.

5. Pemphigus erythematosus (pemphigus seborrhoicus, Senear-Usher syndrome)

This type of pemphigus is very rare. It occurs mainly in women.

The manifestations of the disease appear mainly on places that are exposed to sunlight.

The symptoms appear mainly in seborrheic areas. Hence the name pemphigus seborrhoicus. It occurs mainly on the face, head, chest and back.

Symptoms that appear on the face resemble seborrhoeic dermatitis. Symptoms of the disease include flaccid blisters. The course of the disease may be circumscribed. In some cases, however, it may develop into pemphigus foliaceus.

After the blisters have healed, hyperpigmentation remains.

6. Pemphigus paraneoplasticus

Symptoms of the disease include painful erosions in the oral cavity. They may also appear on the mucosa of the pharynx, larynx, oesophagus, nose and genital mucosa. Paraneoplastic pemphigus is often diagnosed together with a tumour.

In some cases it precedes the tumour. It is often associated with Hodgkin's disease and chronic lymphocytic leukaemia.

The correct diagnosis of pemphigus is based on history, clinical picture, histological examination, immunofluorescence examination and determination of circulating antibodies.

Other routine investigations include blood count, biochemical examination, cancer screening, lung X-ray and bacteriological smears.

The diagnosis is not difficult in most cases. The physician must rule out related chronic blistering skin diseases (bullous diseases).

The following table lists the characteristic features of various blistering diseases

The course of the disease is lengthy.

The initial symptoms of pemphigus are inconspicuous. They arise on any spot of normal, unburned skin by the formation of blisters with clear contents.

When the cover of the blisters ruptures, a red erosion appears, forming a crust. The outer edge of the blister may spread into the surrounding area.

As the disease progresses, the blister eruption continues. An inflammatory rim may form around older blisters. The thin blister cover and its gradual spread to the surrounding area leads to extensive erosions.

More blisters form and the disease spreads further.

How can we prevent worsening progression?

• not eating spicy foods that can irritate the oral mucosa
• drink plenty of fluids
• avoid drinking coffee, alcohol and sugary drinks
• do not smoke
• eat a diet rich in calories and protein
• ensure a higher intake of vitamins
• use mild soaps
• avoid the sun
• follow your doctor's instructions