Abstract
Epidermolysis bullosa acquisita (EBA) is a form of pemphigoid which may come with scarring that looks like dystrophic epidermolysis bullosa hereditaria. This subtype with scarring is named mechanobullous EBA, because blisters are evoked by sudden mechanical trauma, such as hitting the back of the hand to the edge of a table. The other subtype with erythematous lesions without scarring is named inflammatory EBA and may look like bullous pemphigoid. The mucous membranes can be involved in both subtypes. The pathogenesis is mediated by IgG or IgA against type VII collagen, which is the component of anchoring fibrils below the lamina densa. Diagnosis is confirmed by detecting u-serrated linear pattern of immune depositions with direct immunofluorescence microscopy. The pathogenesis of both clinical subtypes is unknown and is not related to binding of a particular epitope of the autoantigen. EBA is associated with systemic lupus erythematosus and colitis ulcerosa. The disease is relative refractory to treatment.
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References
Woodley DT, Briggaman RA, O’Keefe EJ, Inman AO, Queen LL, Gammon WR. Identification of the skin basement-membrane autoantigen in epidermolysis bullosa acquisita. N Eng J Med. 1984;310:1007–13.
Buijsrogge JJ, Diercks GF, Pas HH, Jonkman MF. The many faces of epidermolysis bullosa acquisita after serration pattern analysis by direct immunofluorescence microscopy. Br J Dermatol. 2011;165:92–8.
Terra JB, Jonkman MF, Diercks GF, Pas HH. Low sensitivity of type VII collagen enzyme-linked immunosorbent assay in epidermolysis bullosa acquisita: serration pattern analysis on skin biopsy is required for diagnosis. Br J Dermatol. 2013;169:164–7.
Terra JB, Meijer JM, Jonkman MF, Diercks GF. The n- vs. u-serration is a learnable criterion to differentiate pemphigoid from epidermolysis bullosa acquisita in direct immunofluorescence serration pattern analysis. Br J Dermatol. 2013;169:100–5.
Cunningham B, Kirchmann T, Woodley D. Colchicine for epidermolysis bullosa acquisita. J Am Acad Dermatol. 1996;34:781–4.
Kim JH, Kim SC. Epidermolysis bullosa acquisita. J Eur Acad Dermatol Venereol. 2013;27:1204–13.
Additional Reading
Fine J, Tyring S, Gammon W. The presence of intra-lamina lucida blister formation in epidermolysis acquisita: possible role of leukocytes. J Invest Dermatol. 1989;92:27–32.
Hallel-Halevy D, Nadelman C, Chen M, Woodley DT. Epidermolysis bullosa acquisita: update and review. Clin Dermatol. 2001;19:712–8.
Rusenko K, Gammon W, Fine J, Briggaman R. The carboxyl-terminal domain of type VII collagen is present at the basement membrane in recessive dystrophic epidermolysis bullosa. J Invest Dermatol. 1989;92:623–7.
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Jonkman, M.F. (2016). Epidermolysis Bullosa Acquisita. In: Jonkman, M. (eds) Autoimmune Bullous Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-23754-1_16
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DOI: https://doi.org/10.1007/978-3-319-23754-1_16
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