Disease Directory Pemphigus Vulgaris
Dermatological

Pemphigus Vulgaris

Also known as: PV, autoimmune blistering, desmoglein 3 antibody, mucosal pemphigus

Prevalence

1–5 in 100,000

Onset

Middle age (4th–6th decade)

Type

Autoimmune (non-genetic)

About Pemphigus Vulgaris

Pemphigus vulgaris is a potentially life-threatening autoimmune blistering disorder characterised by circulating IgG autoantibodies directed against desmoglein 3 and, in cutaneous disease, desmoglein 1 — desmosomal cadherins essential for keratinocyte adhesion. Loss of epidermal and mucosal cohesion results in suprabasal acantholysis and flaccid blisters that rupture easily, leaving painful, slow-healing erosions predominantly on mucous membranes and the skin. Without immunosuppressive treatment the condition is chronic and progressive; historically fatal, it now carries significant morbidity from both the disease and its treatments.

Common Clinical Features

Painful oral mucosal erosions, frequently the presenting symptom, preceding skin involvement by months Flaccid, thin-walled blisters on non-inflamed or erythematous skin that rupture rapidly Widespread erosions on the trunk, scalp, face, and flexures that are slow to heal Positive Nikolsky sign: lateral pressure on perilesional skin induces blister extension Oesophageal and pharyngeal erosions causing dysphagia and odynophagia Conjunctival and genital mucosal erosions in severe cases Significant weight loss and nutritional compromise from painful oral and oesophageal involvement

Clinical Trial Eligibility Tips

What to know before applying to Pemphigus Vulgaris trials.

Trials typically require serological confirmation with anti-desmoglein 3 (and/or anti-desmoglein 1) ELISA titres at screening — ensure recent antibody levels are documented within the protocol-specified timeframe.

Disease severity grading using the Pemphigus Disease Area Index (PDAI) is the standard outcome measure; understand your baseline PDAI score as minimum severity thresholds are common eligibility criteria.

Prior rituximab use and timing since last infusion is frequently an exclusion criterion or stratification factor; provide a complete biologic treatment history with infusion dates.

Patient Resources

Patient Organization

International Pemphigus & Pemphigoid Foundation

Visit website ↗

Natural History Registry

IPPF Patient Registry

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:704)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Pemphigus Vulgaris trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Pemphigus Vulgaris. Updated daily.

Related Rare Diseases