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# Pemphigus vulgaris

An autoimmune disease that makes the top skin layer split, causing painful mouth sores and fragile blisters that burst and leave raw areas.

Updated 2026-11-01

## Structures involved

Tap one to see what it is and open it in 3D.

- [Skin](https://durgical.com/en/anatomy/skin/)
- [Gums (gingiva)](https://durgical.com/en/anatomy/gums/)
- [Tongue](https://durgical.com/en/anatomy/tongue/)
- [Buccinator](https://durgical.com/en/anatomy/bucinator/)

## Why it happens

- [Where blisters form: pemphigus vs pemphigoid](https://durgical.com/en/histology/where-blisters-form-pemphigus-vs-pemphigoid/)
- [Cell junctions: tight, adherens, desmosomes, gap, hemidesmosomes](https://durgical.com/en/histology/cell-junctions-tight-adherens-desmosomes-gap-hemidesmosomes/)
- [Skin: epidermis layers, dermis, hypodermis](https://durgical.com/en/histology/skin-epidermis-layers-dermis-hypodermis/)

## What it is

A chronic autoimmune blistering disease in which IgG autoantibodies against desmogleins (the glue proteins between keratinocytes) cause splitting inside the epidermis, just above the basal layer. It almost always involves the oral mucosa and can be life-threatening without treatment.

## Who gets it

Rare, usually starting between 40 and 60 years of age. More frequent in people of Ashkenazi Jewish and Mediterranean ancestry and linked to certain HLA class II types. In Colombia and Latin America the endemic form of pemphigus foliaceus (fogo selvagem) is also known, which must be told apart from pemphigus vulgaris.

## Causes

- IgG autoantibodies against desmoglein 3 (mucosal disease) and often desmoglein 1 (skin disease as well)
- Genetic susceptibility linked to HLA-DR4 and HLA-DR6 haplotypes
- Rarely triggered by drugs containing thiol groups (for example penicillamine or captopril)
- Paraneoplastic pemphigus, a distinct form, associated with lymphoproliferative tumours

## Risk factors

- Middle age
- Ashkenazi Jewish or Mediterranean ancestry
- Specific HLA class II genes
- Exposure to thiol-containing drugs
- Other autoimmune diseases (for example myasthenia gravis or thymoma)

## Pathophysiology

Autoantibodies bind desmogleins in desmosomes, so keratinocytes lose their attachment to each other (acantholysis). The split forms just above the basal layer, whose cells stay attached to the basement membrane like a row of tombstones. Because the blister roof is thin and fragile, blisters rupture easily and leave painful erosions. The pattern of antibodies explains the clinical picture: anti-desmoglein 3 alone gives mucosal disease, and adding anti-desmoglein 1 spreads it to the skin.

## Classification

- Mucosal-dominant pemphigus vulgaris (anti-desmoglein 3)
- Mucocutaneous pemphigus vulgaris (anti-desmoglein 3 and 1)
- Related forms: pemphigus foliaceus (superficial, no mucosal involvement), drug-induced and paraneoplastic pemphigus

## Symptoms and signs

- Painful oral erosions, often the first sign, that may precede skin lesions by months
- Flaccid blisters on normal or red skin that rupture easily, leaving raw, crusted erosions
- Lesions on the scalp, face, [chest](https://durgical.com/en/anatomy/region/chest/), back, armpits and groin
- Positive Nikolsky sign: gentle sideways pressure on normal-looking skin shears off the epidermis
- Pain when eating and swallowing, with weight loss; hoarseness or eye involvement in some patients

## Red flags

- Extensive skin erosions with fluid loss, fever or signs of infection
- Inability to eat or drink because of oral pain, with dehydration
- Signs of sepsis in a patient on immunosuppressive therapy
- Blistering together with an unexplained lymphoma or other tumour (paraneoplastic pemphigus)

## Diagnosis

**Clinical**
Suspected in an adult with persistent oral erosions and flaccid blisters that rupture easily, with a positive Nikolsky sign.

**Tests**
[Skin](https://durgical.com/en/anatomy/skin/) biopsy from the edge of a fresh blister for routine histology (suprabasal acantholysis) and a second biopsy from nearby normal-looking skin for direct immunofluorescence, which shows IgG and C3 in a net-like pattern on keratinocyte surfaces. Serum ELISA for anti-desmoglein 3 and 1 antibodies confirms the diagnosis and helps track activity. Baseline blood tests before immunosuppression.

**Imaging**
Not needed for diagnosis; endoscopy when the oesophagus or larynx is involved, and imaging to look for a tumour if paraneoplastic pemphigus is suspected.

## Differential diagnosis

- Bullous pemphigoid (tense subepidermal blisters, older adults, mucosa usually spared)
- Stevens-Johnson syndrome and toxic epidermal necrolysis
- Erythema multiforme major
- Herpetic gingivostomatitis and recurrent aphthous ulcers
- Oral lichen planus

## Goal of treatment

Stop new blisters, heal erosions, prevent infection and reach remission while keeping the side effects of long-term immunosuppression as low as possible.

## Treatment

**Medical**
Systemic corticosteroids are the first step to control active disease, combined with a steroid-sparing agent.

**Medical**
Rituximab (anti-CD20) is now a first-line option for moderate to severe disease; azathioprine or mycophenolate are used as steroid-sparing drugs; intravenous immunoglobulin for refractory cases.

**Conservative**
Wound care of erosions with non-adherent dressings, gentle oral hygiene and a soft diet; topical corticosteroids for limited lesions.

**Follow-up**
Nursing priorities: fluid balance, pain control, signs of infection, blood glucose, blood pressure and bone health during corticosteroid therapy.

## Complications

- Secondary bacterial or viral infection of erosions and sepsis, the leading cause of death
- Fluid and protein loss from extensive erosions
- Malnutrition and weight loss from painful oral disease
- Adverse effects of long-term corticosteroids and immunosuppressants (diabetes, osteoporosis, infections)

## Prognosis

Before corticosteroids the disease was usually fatal. With modern immunosuppression most patients reach remission, although relapses are common and treatment-related complications now account for much of the morbidity.

## Prevention

- No primary prevention; avoid known trigger drugs in a person with prior drug-induced pemphigus
- Vaccination and screening for latent infections before immunosuppressive therapy
- Bone protection and glucose monitoring during long-term corticosteroid use

## Test yourself

Think of the answer, then tap the question to see it.

## What is the target antigen in pemphigus vulgaris?

Desmoglein 3, often together with desmoglein 1, which are desmosome proteins that hold keratinocytes together.

## Where is the split, and what does histology show?

Intraepidermal, just above the basal layer, with acantholysis and basal cells left in a tombstone row.

## Is the Nikolsky sign positive or negative?

Positive, because the epidermis shears off with light pressure.

## What does direct immunofluorescence show?

IgG and C3 deposited on the surfaces of keratinocytes in a net-like (fishnet) pattern.

## What is the most common cause of death?

Infection and sepsis, from the disease itself or from immunosuppressive therapy.

[Add to my cards (5)](https://durgical.app/estudiar/?add=/en/conditions/pemphigus-vulgaris/)

## Related conditions

- [Bullous pemphigoid](https://durgical.com/en/conditions/bullous-pemphigoid/)
- [Erythema multiforme](https://durgical.com/en/conditions/erythema-multiforme/)
- [Genital herpes (herpes simplex infection)](https://durgical.com/en/conditions/genital-herpes/)
- [Stevens–Johnson syndrome and toxic epidermal necrolysis](https://durgical.com/en/conditions/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis/)
- [Sepsis and septic shock](https://durgical.com/en/conditions/sepsis-and-septic-shock/)
- [Osteoporosis](https://durgical.com/en/conditions/osteoporosis/)

Educational information, not medical advice. For any symptom, see a health professional.

Sources: [Pemphigus Symptoms, Types, Causes, & Risk Factors | NIAMS](https://www.niams.nih.gov/health-topics/pemphigus) (National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), 2026).

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License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/conditions/pemphigus-vulgaris/