Pemphigus erythematosus, also known as Senear-Usher syndrome, is a condition with association with lupus erythematosus (LE) and pemphigus foliaceus characteristics. Pemphigus is shown in interkeratinocyte material by the deposits of acantholysis and immunoglobulin. It is proposed that high doses of UV light are the source of the desmoglein-1 ectodomain cleavage. As in the case of pemphigus foliaceus the circulating anti-desmoglein-1 antibodies precipitate this cleaved off ectodomain along with the membrane zone of the basement, resulting in a lupus band-like look. The lupus portion of pemphigus erythematosus is shown by circulating antinuclear antibodies (ANA) and often by immunoglobulin and by complementing deposits at the dermo-epidermal junction

CAUSES

Patients suffering from pemphigus develop an allergic reaction to desmosomes. The main antigen is desmoglein 1 in patients with pemphigus foliaceus and its variant, pemphigus erythematosus Desmogleins are essential desmosomal proteins for adhesion to keratinocytes. The binding of autoantibodies is postulated to lead to a cascade of intracellular biochemical events that result in the loss of desmosome function.

PATHOPHYSIOLOGY

Patients with pemphigus erythematosus have vesiculobullous or superficially eroded lesions that may ooze and crust, particularly in areas exposed to the sun, such as the face, upper chest and back.

SIGNS AND SYMPTOMS

  • Blisters
  • Rash
  • Itching sensation

COMPLICATIONS

  • Infection of your skin
  • Sepsis
  • Malnutrition

TREATMENT

  • Tetracycline
  • Niacinamide
  • Cyclophosphamide
  • Methotrexate
  • Parenteral gold
  • Hydroxychloroquine
  • Plasmapheresis
  • Mycophenolate mofetil
  • Extracorporeal photochemotherapy
  • Rituximab
  • Dexamethasone-cyclophosphamide combination

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