Understanding Pemphigus Vulgaris

A Comprehensive Guide to This Autoimmune Blistering Disease

Dermatology

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Last updated: Mar 24, 2025

What is Pemphigus Vulgaris?

Pemphigus vulgaris (PV) is a rare autoimmune blistering disorder that affects the skin and mucous membranes. In this condition, the immune system mistakenly attacks proteins that help keep skin cells attached to each other, leading to painful blisters and erosions.

Key Symptoms

  • Painful blisters in the mouth and throat
  • Fragile skin blisters that easily break
  • Erosions on the skin that are slow to heal
  • Scalp involvement in some cases
  • Burning or pain before blisters appear

Risk Factors

  • Age: Usually appears between 40-60 years
  • Genetics: Certain HLA types
  • Jewish or Mediterranean ancestry
  • Some medications can trigger it

Diagnosis

Common Tests Include:

  1. Skin biopsy
  2. Direct immunofluorescence
  3. Blood tests for antibodies
  4. Tzanck smear test

Treatment Options

First-Line Treatments

  • Corticosteroids (oral or topical)
  • Immunosuppressive medications
  • Rituximab in severe cases

Supportive Care

  • Wound care
  • Pain management
  • Infection prevention
  • Nutritional support

Living with PV

Daily Management

  • Careful skin care routine
  • Regular medical follow-up
  • Avoiding trauma to skin
  • Maintaining good oral hygiene

Complications to Watch For

  • Secondary infections
  • Fluid/electrolyte imbalances
  • Medication side effects
  • Difficulty eating or drinking

Prognosis

With proper treatment, most patients achieve remission. Early diagnosis and consistent treatment are crucial for the best outcomes.

When to Seek Help

Contact your healthcare provider if:

  • New blisters develop
  • Signs of infection appear
  • Difficulty eating or swallowing
  • Severe pain
  • Fever develops

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