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Skin blistering is commonly caused by mechanical, physical or infectious insults. More rarely, mutations of structural components of the skin or autoimmunity directed against those structural components lead to skin blistering. Albeit among the less frequent causes of skin blistering, understanding of the ...

Skin blistering is commonly caused by mechanical, physical or infectious insults. More rarely, mutations of structural components of the skin or autoimmunity directed against those structural components lead to skin blistering. Albeit among the less frequent causes of skin blistering, understanding of the pathomechanisms of hereditary and autoimmune skin blistering has provided detailed insights into cutaneous biology and (auto)immunity. In recent years, genetics, definition of autoantigens, model systems and clinical research have led to a tremendous improvement for both diagnosis and treatment for patients suffering from skin blistering diseases. Despite these improvements, however, patient morbidity and mortality remains high. Thus, we herein review the latest developments in pathogenesis, diagnosis and treatment of patients suffering from skin blistering. We hereby aim to increase awareness for these diseases, present the state-of-the art diagnosis and treatment, and (maybe most importantly) stimulate basic, translational and clinical research in the field.

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