What happens in the skin
Epidermal cells are connected to each other by special contacts, which include desmoglein proteins. In pemphigus, the immune system produces antibodies specifically to these proteins. The contacts are destroyed, the cells lose their cohesion, and fluid accumulates between them. The blister forms inside the epidermis, so its cover is very thin and bursts at the slightest touch, often before a person even notices it. Bright red painful erosions remain, which expand at the edges and heal extremely slowly. This distinguishes pemphigus from diseases with a deeper, subepidermal blister.
- Antibodies destroy connections between skin cells
- The bubble forms inside the epidermis, the tire is thin
- Vulgar form - mouth and skin
- Leaf form - skin only, no mouth affected
- The paraneoplastic form is associated with a tumor
- Erosion heals slowly and without scars
Symptoms
For most, the mouth is the first to suffer: painful erosions appear on the mucous membranes of the cheeks, palate, and gums, which interfere with eating, drinking, and brushing your teeth. They are often treated for months as stomatitis without results. After weeks or months, skin manifestations appear: flaccid blisters with cloudy contents on the chest, back, face, scalp, and in folds. They open easily, leaving wet areas with scraps of tires. A characteristic sign is the detachment of apparently healthy skin when lateral pressure is applied near the lesion. Itching is not typical, the leading complaint is pain.
- Painful, non-healing erosions in the mouth
- Difficulty eating and speaking
- Flaccid blisters with a thin covering on the skin
- Weeping erosions with fragments of epidermis
- Skin peeling due to lateral pressure
- Damage to the eyes, nose, and genitals in severe cases
Diagnostics
A biopsy plays a key role: material is taken from a fresh bladder for routine histology and additionally a section of apparently healthy skin next to the lesion for direct immunofluorescence, which reveals antibody deposits between cells. Additionally, antibodies to desmogleins are determined in the blood; their level helps both in diagnosis and in assessing the effectiveness of treatment. A general blood test and biochemistry are performed, sugar levels and bone condition are assessed, since long-term hormonal therapy is ahead. In some forms, the doctor excludes a concomitant tumor.
- Biopsy of a fresh lesion with histology
- Direct immunofluorescence of the skin near the lesion
- Antibodies to desmogleins in the blood
- Complete blood count, glucose, biochemistry
- Examination before long-term hormonal therapy
- Search for a tumor in the paraneoplastic form
Treatment
The basis is systemic glucocorticoids, which quickly suppress the production of antibodies and stop the appearance of new blisters. To reduce the dose of hormones and their side effects, immune suppressants are added. In recent years, for moderate and severe forms, biological therapy aimed at cells that produce antibodies has been widely used. Treatment is always long-term, the regimen is selected and adjusted only by a doctor, and independent withdrawal of drugs leads to severe exacerbation. In parallel, care for erosions, pain relief, soft food and infection prevention are necessary.
- Systemic glucocorticoids as the basis of therapy
- Immune suppressants to reduce hormone doses
- Biological therapy for moderate and severe forms
- Antiseptic treatment of erosions and pain relief
- Soft, non-irritating foods for mouth sores
- Prevention of complications of hormonal therapy, regular monitoring of tests
Living with a disease
Pemphigus requires long-term observation, since remission is achieved gradually, and the dose of drugs is reduced slowly and only under the supervision of a doctor. It is important not to stop treatment when it improves: it is abrupt self-cessation that most often leads to relapse. During therapy, susceptibility to infections increases, so attention to any signs of inflammation and discussion of vaccination with your doctor is necessary. It is worth protecting the skin from injury and friction, using a soft toothbrush, and avoiding spicy, sour and hot foods during an exacerbation.
- Slowly reduce doses only as directed by your doctor
- Inadmissibility of self-discontinuation of drugs
- Regular monitoring of blood tests, sugar, pressure
- Gentle skin and oral care
- Pay attention to signs of infection
- Discussing vaccinations and pregnancy planning with your doctor