Dermatosis ampollares
- Fueyo Casado, A.
- González, M.L.
- López Bran, E.
ISSN: 0304-5412
Ano de publicación: 2018
Título do exemplar: Enfermedades de la piel (II) Psoriasis, pénfigos, toxicodermias y discromías
Serie: 12
Número: 48
Páxinas: 2838-2845
Tipo: Artigo
Outras publicacións en: Medicine: Programa de Formación Médica Continuada Acreditado
Resumo
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.
Referencias bibliográficas
- West J, Fleming KM, Tata LJ, Card TR, Crooks CJ. Incidence and prevalence of celiacdisease and dermatitis herpetiformis in the UK overtwodecades : population basedstudy. Am J Gastroenterol. 2014;109(5): 757-68.
- Bonciani D, Verdelli A, Bonciolini V, D’Errico A, Antiga E, Fabbri P. Dermatitis herpetiformis: from the genetics to the development of skin lesions. Clin Dev Immunol. 2012;2012:239691.
- Alonso-Llamazares J, Gibson LE, Rogers RS 3rd. Clinical, pathologic, and immunopathologic features of dermatitis herpetiformis: review of the Mayo Clinic experience. Int J Dermatol. 2007;46(9):910-9.
- Gammon WR, Heise ER, Burke WA, Fine JD, Woodley DT, Briggaman RA. Increased frequency of HLA-DR2 in patients with autoantibodies to epidermolysis bullosa acquisita antigen: evidence that the expression of autoimmunity to type VII collagenis HLA class II allele associated. J Invest Dermatol. 1988;91(3):228-32.
- Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol. 2011;29(4):432-6.
- Mimouni D, Nousari CH, Cummins DL, Kouba DJ, David M, Anhalt GJ. Differences and similarities among expert opinions on the diagnosis and treatment ofpemphigusvulgaris. J Am Acad Dermatol. 2003;49(6):1059-62.
- Joly P, Maho-Vaillant M, Prost-Squarcioni C, Hebert V, Houivet E, Calbo S; French study group on autoimmune bullous skin diseases. First line rituximab combined with short term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multi centre, parallel group, open label randomised trial. Lancet. 2017; 389(10083):2031-40.
- Fry L, Leonard JN, Swain F, Tucker WF, Haffenden G, Ring N. Long term follow up of dermatitis herpetiformiswith and without dietary glutenwithdrawal. Br J Dermatol. 1982;107(6):631-4.