Pemphigus
Pemphigus is a rare but severe autoimmune disease that affects the skin and mucous membranes and is characterized by blistering and erosions. The disease occurs when the immune system creates antibodies against intercellular proteins (desmogleins) that are responsible for the firm attachment of epidermal cells. This leads to the loss of intercellular connections - a process called acantholysis.
Main forms of pemphigus
1. **Pemphigus vulgaris** – the most common and severe form, with pain and blisters in the oral cavity, pharynx, and skin.
2. **Pemphigus foliaceus** – affects the superficial layers of the epidermis, without involvement of mucous membranes.
3. **Paraneoplastic pemphigus** – associated with underlying malignant diseases (lymphomas, tumors).
Symptoms
- Painful blisters and erosions on the oral mucosa – often the first symptom
- Blisters on the skin – thin-walled, quickly rupture
- Peeling of the epidermis upon light pressure (Nikolsky's sign)
- Erosions and crusts, often on the chest, back, face, scalp
- General malaise, weight loss and risk of infections
When to see a dermatologist?
For painful sores or blisters in the mouth that do not heal
When blisters and erosions appear on the skin without a clear cause
Diagnosis
The diagnosis is made through:
- Clinical examination and history
- Direct immunofluorescence (DIF) – IgG deposition along the border between epidermal cells
- Serological tests – ELISA for anti-desmoglein 1 and 3 antibodies
Treatment
Pemphigus is a chronic disease requiring long-term systemic treatment. Main therapeutic approaches include:
- Systemic corticosteroids (prednisone)
- Immunosuppressants: azathioprine, mycophenolate mofetil, methotrexate
- Biological therapy: **rituximab** – monoclonal antibody against CD20, with very good results in resistant forms
- Local treatment: antiseptic solutions, corticosteroid creams
- Supportive care: oral hygiene, infection prevention, nutrition for oral lesions
In modern medicine, rituximab treatment is considered the gold standard and often leads to a definitive cure. The treatment is expensive, but is fully covered by the National Health Insurance Fund.
Forecast
Before the era of corticosteroids, pemphigus was a fatal disease. Today, with appropriate treatment, most patients achieve disease control or remission. Early diagnosis,
Pemphigus is a serious autoimmune disease, but with timely treatment and specialized care, patients can lead full lives.
Pemphigus is a serious autoimmune disease, but with timely treatment and specialized care, patients can lead full lives.
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Yes – every therapy begins with a mandatory individual consultation and assessment of the condition.
We work both in the field of clinical dermatology and with aesthetic therapies.