Synonyms
Acquired epidermolysis bullosa; Dermolytic pemphigoid; EBA
Definition and Characteristics
An autoimmune subepidermal blistering disease, associated with autoreactivity against collagen VII, a component of anchoring fibrils. The classical mechanobullous presentation of epidermolysis bullosa acquisita (EBA) consists of skin fragility, noninflammatory blistering and healing with scars or milia, but inflammatory disseminated forms resembling the pemphigoid diseases have been described [1].
Prevalence
In Western Europe the annual incidence has been estimated to be 0.25 per million. The prevalence is unclear.
Genes
Susceptibility to develop EBA appears to be increased in Caucasians, Africans and Americans with HLA class II alleles DRB1*1501 and DR5.
Molecular and Systemic Pathophysiology
Collagen VII represents the major component of anchoring fibrils located at the dermoepidermal junction (see Fig. 1, in Bullous pemphigoid). The N-terminal non-collagenous NC1-domain of collagen...
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References
Gammon WR, Briggaman RA, Woodley DT et al. (1984) Epidermolysis bullosa acquisita – a pemphigoid-like disease. J Am Acad Dermatol 11:820–832
Sitaru C, Kromminga A, Hashimoto T et al. (2002) Autoantibodies to type VII collagen mediate Fcγ-dependent γneutrophil activation and induce dermal–epidermal separation in cryosections of human skin. Am J Pathol 161:301–311
Woodley DT et al. (2006) Induction of epidermolysis bullosa acquisita in mice by passive transfer of autoantibodies from patients. J Invest Dermatol 126:1323–1330
Niedermeier A et al. (2007) Clinical response of severe mechanobullous epidermolysis bullosa acquisita to combined treatment with immunoadsorption and rituximab (anti-CD20 monoclonal antibodies). Arch Dermatol 143:192–198
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Hofmann, S., Bruckner-Tuderman, L. (2009). Epidermolysis Bullosa Acquisita. In: Lang, F. (eds) Encyclopedia of Molecular Mechanisms of Disease. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-29676-8_568
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DOI: https://doi.org/10.1007/978-3-540-29676-8_568
Publisher Name: Springer, Berlin, Heidelberg
Print ISBN: 978-3-540-67136-7
Online ISBN: 978-3-540-29676-8
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