Dermatosis ampollares

  1. Fueyo Casado, A.
  2. González, M.L.
  3. López Bran, E.
Aldizkaria:
Medicine: Programa de Formación Médica Continuada Acreditado

ISSN: 0304-5412

Argitalpen urtea: 2018

Zenbakien izenburua: Enfermedades de la piel (II) Psoriasis, pénfigos, toxicodermias y discromías

Saila: 12

Zenbakia: 48

Orrialdeak: 2838-2845

Mota: Artikulua

DOI: 10.1016/J.MED.2018.01.008 DIALNET GOOGLE SCHOLAR

Beste argitalpen batzuk: Medicine: Programa de Formación Médica Continuada Acreditado

Laburpena

Introduction We shall focus on autoimmune bullous disorders in this update. The most common are bullous pemphigoid and pemphigus vulgaris. Epidemiology These are rare diseases but have a major impact on patient's quality of life. The incidence of pemphigoid ranges from 2.8 to 4.28 per 100,000/year, and chiefly presents in people aged over 80 years. Pemphigus vulgaris is rarer and has a more variable geographical distribution. Aetiology/aetiopathogenesis The autoimmune bullous disorders are characterised by the formation of blisters on the skin or mucous membranes. The blisters are caused by the action of auto-antibodies against the skin adhesion proteins. Diagnosis A skin biopsy of the blister is required and another of the skin around it to test for the presence of autoantibodies, as well as their deposit pattern. Prognosis Before the use of corticosteroids, patients with pemphigoid had high rates of mortality. Nowadays complete remission can be achieved in a high percentage of cases, but there is no definitive cure. Treatment Oral steroids are the basic pillar of treatment. They are often associated with corticosteroid-sparing immunosuppressive agents. Rituximab is increasingly used as second line treatment; it induces B-lymphocyte depletion and lasting remission.

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