Universität zu Köln

Lehr, Saskia, Felber, Felicitas, Tantcheva-Poor, Iliana, Kessler, Christina, Eming, Ruediger, Nystroem, Alexander, Rizzi, Marta and Kiritsi, Dimitra (2022). Occurrence of autoantibodies against skin proteins in patients with hereditary epidermolysis bullosa predisposes to development of autoimmune blistering disease. Front. Immunol., 13. LAUSANNE: FRONTIERS MEDIA SA. ISSN 1664-3224

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Abstract

Skin blistering disorders are associated with inherited defects in proteins involved in the dermal-epidermal adhesion or autoantibodies targeting those proteins. Although blistering in hereditary epidermolysis bullosa (EB) is pathogenetically linked to genetic deficiency of distinct proteins of the epidermis or the dermal-epidermal junction, circulating autoantibodies against these proteins have also been identified in EB patients. So far, autoantibodies have been considered bystanders in EB and active pathogenicity of them in EB has not been disclosed. In sera of a cohort of 258 EB patients, we found by ELISA in 22% of the patients autoantibodies against the bullous pemphigoid antigen BP180. The titers correlated negatively with collagen VII skin expression and positively with disease severity. Among those patients, we identified six (2.33%) with clinical features of an autoimmune bullous disorder (AIBD) and positive indirect immunofluorescence (IIF) staining. In literature, we found four more cases of EB patients developing disease-aggravating AIBD. Co-existence of these two rare skin disorders suggests that EB patients have a predisposition for the development of AIBD. Our work highlights that EB patients with increased itch or blister formation should be evaluated for additional AIBD and repeated screening for changes in autoantibody titers and skin-binding specificities is advised.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Lehr, Saskia UNSPECIFIED UNSPECIFIED UNSPECIFIED Felber, Felicitas UNSPECIFIED UNSPECIFIED UNSPECIFIED Tantcheva-Poor, Iliana UNSPECIFIED UNSPECIFIED UNSPECIFIED Kessler, Christina UNSPECIFIED UNSPECIFIED UNSPECIFIED Eming, Ruediger UNSPECIFIED UNSPECIFIED UNSPECIFIED Nystroem, Alexander UNSPECIFIED UNSPECIFIED UNSPECIFIED Rizzi, Marta UNSPECIFIED UNSPECIFIED UNSPECIFIED Kiritsi, Dimitra UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-679395
DOI:	10.3389/fimmu.2022.945176
Journal or Publication Title:	Front. Immunol.
Volume:	13
Date:	2022
Publisher:	FRONTIERS MEDIA SA
Place of Publication:	LAUSANNE
ISSN:	1664-3224
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language ACQUISITA Multiple languages Immunology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/67939