Pemphigus – causes, symptoms, diagnosis. Treatment of common and deciduous pemphigus

Proven content

Pemphigus is an autoimmune disease of the skin, the characteristic symptom of which are blisters prone to cracking and turning into erosions. There are common pemphigus, erythematous and paraneoplastic pemphigus. Pemphigus is treated with steroids and immunosuppressants, the therapy is long-term.

Definition of pemphigus

Pemphigus is a rare skin ailment that is classified as an autoimmune disease, i.e. diseases whose essence is the production of antibodies directed at the body’s own cells. A characteristic symptom of the disease are intra-epidermal blisters, which tend to burst and turn into painful erosions. The disease mainly affects adults and is treated with steroids or immunosuppressants.

Pemphigus – causes

The immune system produces antibodies to protect the body against the harmful effects of bacteria and viruses. In the course of pemphigus, these antibodies are treated by the immune system as foreign. The reason for this is the formation of blisters on the skin and mucous membranes.

In fact, the causes of this autoimmunity are not fully known, and therefore idiopathic pemphigus of unknown cause is often diagnosed. Doctors suspect that pemphigus may have a genetic component. Patients previously diagnosed with other autoimmune diseases have a greater predisposition to developing the disease. In addition, the use of certain preparations may increase the risk of ailments, e.g. non-steroidal anti-inflammatory drugs, ACE-inhibitors or penicillins. Eating certain foods and exposing yourself to the sun for a long time also increases your chances of getting pemphigus.

Pemphigus symptoms

The symptoms of pemphigus can be observed as erosions on the mucous membranes (mouth, conjunctiva, esophagus) and blisters on the scalp, neck, trunk and armpits. Erosions and blisters cause severe pain and may be accompanied by a fever with chills. Over time, bullous lesions appear on the mucous membranes of the digestive tract and respiratory tract.

The following types of pemphigus are distinguished:

1. pemphigus vulgaris (the most common variety),
2. deciduous pemphigus,
3. seborrheic pemphigus,
4. pemphigus erythematosus,
5. paraneoplastic pemphigus.

Symptoms of common pemphigus

Pemphigus vulgaris is the most common type and has the most severe course. The formation of blisters is usually preceded by erosions on the oral mucosa, and even around the larynx and esophagus (difficulty swallowing) or the genitals and conjunctiva. Common pemphigus also has its own varieties, which include:

1. rocking pemphigus – a rare type, characterized by warty and overgrown foci in the area of ​​articular bends and body openings;
2. herpetic pemphigus – skin lesions have a round shape;
3. Brazilian pemphigus – occurs mainly in South America, and its genesis lies in viral infection transmitted by insects. It is an epidemic variety.

Symptoms of deciduous pemphigus

In leafy pemphigus, the blisters do not attack the mucous membranes. The skin lesions are extensive, with general dermatitis or atrophic changes in skin appendages (e.g. nails). Among the varieties of deciduous pemphigus we can distinguish: erythematous; seborrheic; herpetic and paraneoplastic.

Paraneoplastic pemphigus – most often accompanies neoplasms, its cause is probably, as in other cases, the formation of autoantibodies due to allergy to tumor cells. This type of pemphigus is much more severe, in addition, the symptoms of the mucous membranes are more painful, and erosions are formed even in the gastrointestinal tract and respiratory system. A serious complication of paraneoplastic pemphigus is bronchiolar obstructive disease.

Who gets pemphigus most often?

Pemphigus most often affects adults, usually the onset of the disease occurs between the ages of 30 and 60. As for children – they get sick quite rarely. Gender does not matter here, pemphigus affects women as much as men. In contrast, people with a history of other autoimmune diseases and those with a family history of pemphigus are at increased risk.

In addition, cancer patients may develop paraneoplastic pemphigus, especially in the course of lymphoma or thymoma.

Pemphigus – diagnosis

The diagnosis of pemphigus disease consists primarily in conducting a medical interview with the patient and a physical examination. Sometimes also histopathological examinations of the skin section are performed in order to find or exclude the presence of autoantibodies directed against typical antigens. A physician diagnosing pemphigus should rule out a possible paraneoplastic syndrome (related to cancer).

Pechosis – treatment

In the treatment of pemphigus, constant and pulse treatment regimens are used, which are based on the use of steroids (prednisone, dexomethasone) and immunosuppressants that inhibit the production of antibodies (azathioprine, cyclophosphamide). Intravenous immunoglobulins are used less frequently. In the case of infection of the lesions, antibiotics are used. Antifungal, antibacterial and disinfecting preparations, including ointments with glucocorticosteroids, are recommended for topical application.

Endemic pemphigus is treated with antimalarial preparations, while in the paraneoplastic variant it is important to treat the neoplasm itself (remove the tumor). In the treatment of paraneoplastic pemphigus, other treatments typical of other types of the disease are usually not used, as they may increase the aggressiveness of the cancer.

Pemphigus is a chronic condition that is very troublesome for the patient, but rarely causes death. The worst prognosis is in neoplastic pemphigus and the best in pemphigus.

The best internist in your area – make an appointment