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Pemphigus vulgaris

Pemphigus vulgaris · Pemfigus vulgaris

What it is

Antibodies cut the glue between skin cells, so blisters form in the mouth and on the skin and burst into raw erosions; once fatal, now controllable.

Symptoms & signs

  • Painful mouth sores that do not heal
  • fragile blisters that burst easily
  • raw weeping patches
  • skin peeling on light rubbing
  • pain on eating

🚩 Get help if…

  • call 112 / 911 now Fever, shivers or widespread raw skin
  • call 112 / 911 now Cannot drink or swallow because of mouth sores
  • see a doctor soon Blisters spreading despite treatment
  • see a doctor soon Painful, crusted or oozing sores — possible infection

Where it strikes

Medical treatment

  • Skin biopsy with immunofluorescence
  • high-dose steroids
  • rituximab (first line now)
  • azathioprine or mycophenolate
  • wound and mouth care, infection control

Natural & lifestyle

  • Soft bland diet during flares
  • gentle skin care, no friction
  • calcium and vitamin D while on steroids

Emerging & experimental

  • FcRn blocker efgartigimod — the phase 3 ADDRESS trial missed its goal not confirmed in trials
  • CAAR-T cells targeting the rogue B cells
  • BTK inhibitor rilzabrutinib — the phase 3 PEGASUS trial missed its goal not confirmed in trials

Alternative, off-label & anecdotal

Approaches used outside the guidelines, with what their supporters report and what testing has found so far.

  • Nicotinamide with tetracycline — used for mild disease; small studies early / small studies
  • Aloe vera mouth gels — soothing; untested anecdotal / traditional
  • Low-thiol diets (no garlic, onion, leek) — anecdotal; thiols can trigger it in the lab anecdotal / traditional

ICD codes