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Brunsting-Perry cicatricial pemphigoid

What is Brunsting-Perry? scar pemphigoid?

Brunsting-Perry cicatricial pemphigoid (or mucous membrane pemphigoid) is a rare variant of mucous membrane pemphigoid It is a autoimmune blistering skin condition located a la región de la cabeza y el cuello. La característica más llamativa es el desarrollo de cicatrices significativas. A diferencia del clásico pemphigoid de la membrana mucosa, la afectación de la membrana mucosa es menos común y no se asocia con cicatrices.

Some authors believe that Brunsting-Perry scarring pemphigoid is better known as the Brunsting-Perry variant of acquired epidermolysis bullosa. Acquired epidermolysis bullosa is another rare disorder of autoimmune blistering.

What causes Brunsting-Perry scarring pemphigoid?

Brunsting-Perry scarring pemphigoid is an autoimmune disorder. This means that an individual's immune system begins to react against his own tissue. In this case, autoantibodies react with the components of the basement membrane, particularly the anchorage filament laminin component 5, and the hemidesmosome-BP180 and BP230 associated proteins. In some patients antibodies to collagen VII have been described.

In 20 to 30% of patients with Brunsting-Perry scarring pemphigoid, skin antibodies can be detected in their bloodstream. (indirect positive immunofluorescence)

Clinical presentation of Brunsting-Perry cicatricial pemphigoid

The diagnosis of Brunsting-Perry scarring pemphigoid is based on the following characteristics:

  • Itch Urticated license plate located on the head and neck ("urticated" means a bump-like a plate similar to urticaria
  • The plaque enlarges for weeks or months.
  • The blisters arise around the edge of the plate.
  • Occasionally a smaller secondary plate appears.
  • The plaque heals slowly with significant scarring over months or years.
  • Injuries to the scalp are associated with scarring. alopecia (hair lost)
  • Mucosal membrane lesions can occur months after primary injury
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Biopsy the characteristics are similar to bullous pemphigoid, but without scars.

What is the treatment for Brunsting-Perry scarring pemphigoid?

The treatment of Brunsting-Perry scarring pemphigoid is similar to that of classic scarring pemphigoid. The goal of treatment is to promote healing.

Ultra powerful current steroids such as clobetasol propionate may suffice as monotherapy, however many patients require systemic Corticosteroids Other immunomodulatory agents used for Brunsting-Perry cicatricial pemphigoid have included:

  • Methotrexate
  • Dapsone
  • Excision with a full-thickness skin graft

In general, patients with Brunsting-Perry scarring pemphigoid have better forecast than patients with classic scarring pemphigoid or acquired epidermolysis bullosa.