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Pemphigus Vulgaris

An autoimmune blistering disease causing painful intraepidermal blisters and erosions.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dermatoloji department. Book Appointment →

What is Pemphigus Vulgaris?

Pemphigus vulgaris (PV) is the most common form of pemphigus, characterized by IgG autoantibodies that target desmoglein-3, a desmosomal protein essential for keratinocyte adhesion. Loss of cell-cell connection produces acantholysis and intraepidermal blistering.

The disease typically affects adults aged 40–60 and is more common in people of Mediterranean, Middle Eastern, Ashkenazi Jewish, and South Asian descent. Untreated PV can be fatal due to fluid loss, sepsis, and sequelae of large erosions.

Diagnosis is confirmed by a combination of clinical examination, lesional skin biopsy showing suprabasal acantholysis, direct immunofluorescence demonstrating intercellular IgG and C3 deposits, and ELISA detection of anti-desmoglein-3/1 antibodies.

Symptoms

Painful oral erosions (often the first sign) on buccal mucosa, palate, gingiva
Flaccid blisters on the trunk, scalp, face, and intertriginous areas that rupture easily
Painful, slow-healing erosions and crusted lesions
Positive Nikolsky sign (lateral pressure causes epidermal sliding)
Esophageal, conjunctival, nasal, or genital mucosal involvement
Weight loss due to painful eating, secondary bacterial infection

Risk Factors

Genetic predisposition (HLA-DRB1*04:02, DRB1*14:01, DQB1*05:03)
Ethnicity: Ashkenazi Jewish, Mediterranean, Middle Eastern, South Asian
Drug-induced PV (penicillamine, captopril, NSAIDs, rifampin)
Coexisting autoimmune disease (myasthenia gravis, thymoma, lupus)
Stress, viral infection, ultraviolet exposure as possible triggers

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent painful mouth sores not healing within 2–3 weeks
  • New flaccid blisters or unexplained erosions on the skin
  • Difficulty eating or drinking due to oral pain
  • Rapidly enlarging skin lesions with crusting and weeping

Treatment Methods

01
Rituximab (anti-CD20 monoclonal antibody) — first-line for moderate-severe disease per current guidelines
02
Systemic corticosteroids (prednisolone 0.5–1.5 mg/kg/day) tapered as remission is achieved
03
Steroid-sparing immunosuppressants: azathioprine, mycophenolate mofetil, cyclophosphamide
04
IVIG or plasmapheresis for severe refractory cases
05
Topical care: gentle wound dressings, antiseptics, oral analgesics, antimicrobial mouthwash
06
Bone protection (calcium, vitamin D, bisphosphonates), PCP prophylaxis, infection screening on long-term immunosuppression
07
Long-term follow-up to monitor relapse, antibody titers, and treatment toxicity

Which Department to Visit?

You can visit our Dermatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.