Chapter 74

Autoimmune Bullous Diseases

Nina van Beek

Nina van Beek

Department of Dermatology, University of Lübeck, Germany

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Enno Schmidt

Enno Schmidt

Department of Dermatology, University of Lübeck, Germany

Lübeck Institute of Experimental Dermatology (LIED), Lübeck, Germany

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First published: 20 November 2019
Citations: 3

Summary

Autoimmune blistering diseases (AIBDs) are characterized by tissue-bound and circulating autoantibodies against proteins of epidermal/epithelial desmosomes in pemphigus diseases and proteins of the dermal–epidermal junction in pemphigoid disorders. AIBDs are difficult to distinguish based on clinical presentation alone, in particular in infants and children. Modern diagnosis of AIBDs depends on the clinical picture, direct immunofluorescence microscopy and detection of serum autoantibodies. Treatment of childhood AIBDs is challenging due to the lack of controlled clinical trials and involves a multidisciplinary approach including dermatologists, paediatric dermatologists and paediatricians. Here, essential epidemiological, clinical and immunopathological findings as well as treatment regimens in pemphigus and pemphigoid diseases in a paediatric population are presented.

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