Pemphigus Vulgaris

Peptimmune is developing PI-0824 peptide immunotherapy for pemphigus vulgaris.

Disease Overview -Pemphigus vulgaris is an autoimmune disorder caused by the formation of auto-antibodies to desmoglein 3, a protein found in normal healthy skin. The antibodies produce a reaction that leads to separation of the epidermal (skin) cells. Those afflicted suffer from burn-like lesions and blisters all over their body.

Incidence

Things you see in a Lab
  • It is estimated that 30-50,000 people have pemphigus vulgaris worldwide with annual new cases diagnosed at a rate of 5 per one hundred thousand.
  • It is known to affect people across racial and cultural lines, with certain groups of people (such as Eastern European Jews, people of Mediterranean, Northern Indian, and Persian descent) who have a higher incidence of the disease.
  • Pemphigus vulgaris is associated with a mutation in MHC II HLA DRß1*0402 and is seen with more prevalence among Jewish people of Ashkenazi descent.

Symptoms

  • Patients may present with painful ulcerations of the mouth that are commonly misdiagnosed as simple mouth sores that become widespread and may appear all over the body.
  • The skin lesions are commonly misdiagnosed as an infectious disease - bacterial or fungal.
  • The diagnosis of pemphigus vulgaris is eventually made by biopsy and immunofluorescence.
  • If left untreated, the blisters can lead to devastating infections, discomfort, and ultimately death from sepsis.
  • The course of the disease is almost always relentless and progressive.

Current treatment

  • Current treatments include corticosteroid and chemotherapeutic immunosuppressive drugs.
  • Results in serious and difficult-to-manage side effects that may lead to discontinuation of therapy.
  • The most common cause of death is from complications of the treatment.
  • There is no therapy specifically aimed at the treatment of pemphigus vulgaris.
Peptimmune Development

For more information about pemphigus vulgaris, please visit www.pemphigus.org

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