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Pemphigus Vulgaris (Detailed)

Severe autoimmune intraepidermal blistering disease against desmoglein-3 and desmoglein-1.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

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 (Detailed)?

Pemphigus vulgaris (PV) is an autoimmune mucocutaneous disorder caused by IgG autoantibodies against desmoglein-3 (mucosal type) and additionally desmoglein-1 in mucocutaneous involvement.

It manifests as painful, slow-healing oral erosions, flaccid skin bullae and erosions, and a positive Nikolsky sign. Untreated disease has historically high mortality from sepsis and dehydration.

Diagnosis combines clinical findings, perilesional biopsy showing intraepidermal acantholysis, direct immunofluorescence (intercellular IgG deposits) and serum ELISA for anti-Dsg3/1 antibodies.

Symptoms

Painful oral and pharyngeal erosions
Flaccid skin bullae rupturing easily
Positive Nikolsky sign
Erosions on scalp, face, axilla and groin
Difficulty eating and weight loss
Genital, conjunctival or nasal involvement
Hoarseness from laryngeal involvement

Risk Factors

HLA-DR4 and HLA-DR14 alleles
Mediterranean, Jewish or South Asian ancestry
Drug triggers: thiols, ACE inhibitors, penicillamine
Coexisting autoimmune disease (myasthenia, thymoma)
Middle-aged adults

When to See a Doctor?

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

  • Painful, persistent oral ulcers >2 weeks
  • Skin bullae rupturing into widespread erosions
  • Inability to eat or drink
  • Signs of infection: fever, purulence
  • Steroid-tapering relapse

Treatment Methods

01
Rituximab (anti-CD20) — first-line in moderate-to-severe disease
02
Systemic corticosteroids for induction
03
Steroid-sparing immunosuppressants (azathioprine, mycophenolate)
04
IVIG or immunoadsorption in refractory cases
05
Wound care, oral hygiene and analgesia
06
Long-term follow-up with anti-Dsg3 titers

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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Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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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.