Autoimmune Bullous Diseases
Nina van Beek
Department of Dermatology, University of Lübeck, Germany
Search for more papers by this authorEnno Schmidt
Department of Dermatology, University of Lübeck, Germany
Lübeck Institute of Experimental Dermatology (LIED), Lübeck, Germany
Search for more papers by this authorNina van Beek
Department of Dermatology, University of Lübeck, Germany
Search for more papers by this authorEnno Schmidt
Department of Dermatology, University of Lübeck, Germany
Lübeck Institute of Experimental Dermatology (LIED), Lübeck, Germany
Search for more papers by this authorPeter Hoeger
Search for more papers by this authorVeronica Kinsler
Search for more papers by this authorAlbert Yan
Search for more papers by this authorJohn Harper
Search for more papers by this authorArnold Oranje
Search for more papers by this authorChristine Bodemer
Search for more papers by this authorMargarita Larralde
Search for more papers by this authorVibhu Mendiratta
Search for more papers by this authorDiana Purvis
Search for more papers by this authorSummary
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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