ConditionSkin, hair & nails
Pemphigus
Pemphigus is a rare autoimmune disease in which the immune system attacks proteins (desmogleins) that hold skin cells together. The skin and mucous membranes then form fragile blisters that burst easily, leaving painful raw areas. The most common type, pemphigus vulgaris, usually starts with mouth sores. Pemphigus foliaceus affects only the skin, with crusted, scaly sores. It usually begins in middle age. Without treatment it can be life-threatening, but modern treatment, especially rituximab, controls it in most people.
Also called: Pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus, autoimmune blistering disease
At a glance
- What it is
- Autoimmune blistering of the skin and mucous membranes.
- Main types
- Pemphigus vulgaris (mouth and skin), pemphigus foliaceus (skin only), paraneoplastic pemphigus (with cancers).
- Hallmarks
- Fragile blisters that rupture easily; painful mouth erosions; skin that shears with rubbing.
- Diagnosis
- Skin biopsy with direct immunofluorescence and blood antibody tests.
- Treatment
- Rituximab, corticosteroids and other immunosuppressants; wound and mouth care.
- Outlook
- Most achieve remission with treatment; ongoing monitoring needed.
When to get help
Call 911 or go to the ER
- Rapidly spreading blisters or raw skin over large areas, fever, chills or feeling very unwell (risk of infection and fluid loss)
- Inability to eat or drink because of mouth and throat sores, or signs of dehydration
See a doctor within a day or two
- New painful mouth sores lasting more than 2 weeks, with or without skin blisters
- Blisters or sores that become red, hot, crusted yellow or pus-filled (possible infection)
- Fever, cough or new infections while taking immune-suppressing medicines
Book a routine appointment
- Recurring or persistent blisters or crusted sores on the scalp, face, chest or back
- Eye irritation, hoarseness or genital sores alongside mouth sores
What happens in pemphigus
Skin cells in the outer layer (epidermis) are linked by connections called desmosomes. In pemphigus, antibodies attack desmosome proteins called desmoglein 3 (mainly in mucous membranes) and desmoglein 1 (mainly in the skin). The cells separate (acantholysis), and blisters form within the epidermis. Because the blister roof is thin, blisters break easily, leaving sore, raw areas.
Types
| Type | Main features |
|---|---|
| Pemphigus vulgaris | Most common; painful mouth sores often first, then flaccid skin blisters and erosions |
| Pemphigus foliaceus | Skin only; crusted, scaly sores on scalp, face, chest and back; no mouth sores |
| Paraneoplastic pemphigus | Severe mouth sores and varied skin rash with an underlying tumor, often lymphoma, leukemia, Castleman disease or thymoma; can affect the lungs |
| Drug-induced pemphigus | Triggered by medicines such as penicillamine or ACE inhibitors |
| IgA pemphigus | Rare; small pus-filled blisters |
Symptoms
Pemphigus vulgaris
- painful sores in the mouth, often for weeks or months before skin involvement; may be mistaken for mouth ulcers or gum disease
- difficulty eating and swallowing; hoarseness
- soft, fragile blisters on the skin that burst, leaving painful raw areas that heal slowly
- sores in the eyes, nose, throat or genitals
- Nikolsky sign: rubbing normal-looking skin causes the top layer to slide off
Pemphigus foliaceus
- crusted, scaly, sometimes itchy sores, often starting on the scalp, face and upper body
- blisters may be too superficial to see intact
Who gets it
Pemphigus usually starts between ages 40 and 60. Pemphigus vulgaris is more common in people of Ashkenazi Jewish, Mediterranean, Middle Eastern and South Asian descent. A form of pemphigus foliaceus (fogo selvagem) is endemic in parts of Brazil. Specific HLA gene types increase susceptibility.
Pemphigus vs bullous pemphigoid
| Pemphigus | Bullous pemphigoid | |
|---|---|---|
| Blister level | Within the epidermis | Below the epidermis |
| Blisters | Fragile, rupture easily | Tense, stay intact |
| Mouth involvement | Common (vulgaris) | Uncommon |
| Typical age | 40 to 60 | Over 70 |
| Itch | Usually pain | Often intense itch |
Diagnosis
- Skin biopsy: shows separation within the epidermis.
- Direct immunofluorescence: a biopsy from skin next to a blister shows antibodies deposited between skin cells. This is key.
- Blood tests: antibodies to desmoglein 1 and 3 by ELISA, which also help monitor disease activity.
- Further tests if paraneoplastic pemphigus is suspected: imaging for an underlying tumor.
Treatment
Treatment is led by a dermatologist and aims to stop new blisters, heal sores and achieve remission with the lowest effective medicines.
- Rituximab: an infusion that depletes the B cells making the antibodies. It is first-line for moderate to severe pemphigus vulgaris and leads to remission in most people.
- Corticosteroids: prednisone controls disease quickly; used at the start and tapered.
- Steroid-sparing immunosuppressants: mycophenolate, azathioprine.
- Other options for difficult cases: intravenous immunoglobulin (IVIG), plasma exchange.
- Topical treatments: strong steroid creams and mouth rinses for local sores.
Supportive care
- gentle skin care and non-stick dressings for open areas
- soft, bland foods; avoid spicy, acidic and crunchy foods
- good dental hygiene with a soft toothbrush; regular dental reviews
- pain relief, including numbing mouthwashes before meals
- watching for infection and bone health and blood sugar effects of steroids
- vaccinations (before rituximab where possible)
Complications
- skin infections and sepsis
- dehydration and malnutrition from mouth sores
- side effects of long-term steroids and immunosuppression: infections, osteoporosis, diabetes, weight gain
- scarring is uncommon, but skin color changes can persist for months
Outlook
Before steroids, pemphigus vulgaris was usually fatal. Today, most people achieve control, and many reach complete remission and can stop treatment, especially after rituximab. Relapses can occur and are treated again. Paraneoplastic pemphigus has a more serious outlook.
Frequently asked questions
What is pemphigus?
Pemphigus is a rare autoimmune disease in which the immune system attacks proteins that hold skin cells together, causing fragile blisters and painful sores on the skin and in the mouth, throat, eyes or genitals.
What does pemphigus look like?
Pemphigus vulgaris usually starts with painful mouth sores, followed by soft blisters on the skin that break easily and leave raw, painful patches. Pemphigus foliaceus causes crusted, scaly sores on the scalp, face and upper body without mouth sores.
Is pemphigus contagious?
No. Pemphigus is an autoimmune disease and cannot spread from person to person. However, open sores can become infected with bacteria or viruses, which is why wound care matters.
Can pemphigus be cured?
There is no permanent cure, but many people achieve complete remission and can stop treatment, especially after rituximab. Relapses can occur and are usually treatable. Long-term follow-up with a dermatologist is important.
Sources
This guide was written from current patient and clinical guidance, including:
- American Academy of Dermatology: Pemphigus, diagnosis and treatment
- International Pemphigus and Pemphigoid Foundation: About pemphigus
- National Institute of Arthritis and Musculoskeletal and Skin Diseases: Pemphigus
- European Academy of Dermatology and Venereology: Guideline for diagnosis and treatment of pemphigus
- MedlinePlus: Pemphigus vulgaris