Pemphigus vulgaris
Pemphigus vulgaris is a rare autoimmune condition in which the immune system destroys the connections between cells within the upper layer of the skin and…
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Medicines commonly prescribed for Pemphigus vulgaris
For informational purposes only. Always consult a doctor before using any medicine.
Dosage form: ORAL SOLUTION/SUSPENSION DROPS, 13.3 mg prednisolone estragelate/mlActive substance: prednisoloneManufacturer: Laboratorios Sonphar S.L.Prescription requiredDosage form: TABLET, 100 mgActive substance: azathioprineManufacturer: Dr. Falk Pharma GmbhPrescription requiredDosage form: ORAL SOLUTION/SUSPENSION, 4 mg/mlActive substance: prednisoloneManufacturer: Laboratorio Aldo Union S.L.Prescription required
Pemphigus vulgaris is a rare autoimmune condition in which the immune system destroys the connections between cells within the upper layer of the skin and mucous membranes. Flaccid blisters form that rupture easily, leaving painful erosions. Unlike bullous pemphigoid, pemphigus more often begins in the mouth and follows a far more serious course, so treatment needs to be rapid and decisive.
Symptoms
- onset in the mouth: in most people the first sign is painful erosions on the lining of the cheeks, palate, tongue and gums. This is often the only feature for months and is mistaken for mouth ulcers;
- pain on eating and swallowing, with consequent weight loss;
- flaccid blisters on the skin with clear contents and a thin roof, rupturing at the slightest touch;
- painful, weeping erosions that heal poorly, with crusting at the edges;
- typical skin sites: scalp, face, chest, back, armpits and groin;
- pain predominates over itching — this distinguishes it from pemphigoid;
- the nose, throat, oesophagus, eyes and genitals may be involved;
- Nikolsky's sign — gently shearing the skin next to an erosion causes the top layer to peel away. Do not test this yourself.
When to see a doctor
This is not a condition to wait out.
- mouth erosions have not healed in 2–3 weeks;
- blisters that rupture easily or weeping erosions have appeared on the skin;
- eating and drinking are painful and you are losing weight;
- a large area is affected;
- there is fever, the erosions have become purulent and the surrounding skin is hot — possible sepsis, requiring emergency care;
- you develop eye discomfort, difficulty swallowing or hoarseness.
How it is diagnosed
The diagnosis is confirmed by biopsy: a sample of skin or mucosa including the edge of a lesion is taken. Routine histology and immunofluorescence are performed, the latter showing antibodies between the cells of the upper layer. A blood test for antibodies to desmogleins is also done, and their level is used to monitor treatment later.
It is important not to confuse it with bullous pemphigoid, erythema multiforme, severe drug reactions and aphthous stomatitis — treatment differs in each case.
Causes
Antibodies attack desmosomal proteins, which hold skin cells together. The cause is unknown. Contributing factors include:
- genetic predisposition — the condition is more common in certain ethnic groups;
- medicines — some blood pressure drugs and penicillin-class antibiotics;
- other autoimmune conditions;
- age 40–60, the typical age of onset;
- rarely, tumours, which produce a distinct form of the disease.
Treatment
The aim is to stop new blisters quickly and then reduce treatment to the minimum necessary. Untreated, the condition is life-threatening; with treatment the outlook is generally good.
- oral corticosteroids at an adequate dose — the basis of initial treatment;
- rituximab, a biologic agent; it is now used early, often alongside steroids from the outset, because it allows the steroid dose to be reduced sooner and produces lasting remissions;
- immunosuppressants such as azathioprine or mycophenolate;
- intravenous immunoglobulin and plasma exchange in severe disease;
- care of the erosions — non-adherent dressings, gentle antiseptics and pain relief;
- mouthwashes and gels, with a topical steroid where needed;
- antibiotics if infection is added;
- bone and stomach protection and monitoring of blood pressure and glucose — essential with long-term steroids.
Treatment lasts a long time — months and years — with a gradual dose reduction. Changing or stopping it yourself is dangerous: abrupt withdrawal causes a severe flare.
What you can do
- do not burst blisters, and dress the erosions as your doctor has shown you;
- soft, cool food without anything spicy, acidic, hard or hot; drink through a straw if it hurts;
- keep an eye on your weight: with mouth erosions it is easy to eat too little;
- a soft toothbrush, thorough but gentle oral hygiene and regular dental review;
- avoid friction, tight clothing, skin trauma and excessive sun;
- do not smoke;
- keep a list of all your medicines and do not add new ones without checking;
- follow the vaccination schedule agreed with your doctor: treatment suppresses immunity.
Common questions
Is it contagious? No.
Can it be cured? Stable remission is achievable and many people are able to stop treatment. Monitoring continues.
Will it leave scars? The skin usually retains dark patches that fade. Scarring is possible with deep erosions and with eye involvement.
Why such a high steroid dose? To halt the process quickly. The dose is then reduced, and that is precisely why the other drugs are added.
Online consultation
In an online consultation the doctor reviews photographs, helps distinguish pemphigus from mouth ulcers and from pemphigoid, explains which tests confirm the diagnosis, and advises on wound care and eating until you are seen by a dermatologist. With this picture it is best not to delay.
This material is for information only and does not replace a medical consultation.
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Reviewed on Aug 3, 2026
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