Pemphigus

๐Ÿงช Pemphigus

Pemphigus is a rare, serious autoimmune blistering disorder that causes painful sores and blisters on the skin and mucous membranes (such as the mouth, nose, throat, and genitals).


๐Ÿ” Causes

  • Autoimmune attack on desmogleins (proteins that help skin cells stick together)
  • The immune system mistakenly produces antibodies that break down cell adhesion, causing blisters
  • Triggered by:
    • Genetics
    • Certain medications (e.g., penicillamine, ACE inhibitors)
    • Sometimes idiopathic (no clear cause)

โš ๏ธ Types of Pemphigus

  1. Pemphigus vulgaris (most common and severe):
    • Painful oral and skin blisters
    • Starts in mucous membranes
  2. Pemphigus foliaceus:
    • More superficial skin involvement
    • Rarely involves the mouth
  3. Paraneoplastic pemphigus:
    • Associated with underlying cancers

๐Ÿงช Symptoms

  • Fragile blisters that rupture easily
  • Raw, painful skin erosions
  • Blisters inside the mouth, nose, throat, or genitals
  • Crusting and oozing
  • Delayed healing and risk of infection

๐Ÿงซ Diagnosis

  • Skin biopsy with direct immunofluorescence
  • Blood tests for anti-desmoglein antibodies
  • ELISA test for specific antibody levels

๐Ÿ› ๏ธ Treatment

  • Corticosteroids (topical or systemic) โ€“ first-line treatment
  • Immunosuppressants (e.g., azathioprine, mycophenolate)
  • Biologics such as rituximab (for severe or refractory cases)
  • Antibiotics or antifungals for secondary infections
  • Wound care and pain control

๐Ÿ›ก๏ธ Tips

  • Avoid trauma to the skin
  • Use gentle skin and oral care products
  • Regular follow-up with dermatology/immunology team
  • Nutritional support if oral lesions impair eating
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