Autoimmune dermatoses, bullous

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Definition
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Blistering autoimmune diseases with subepidermal or intraepithelial blistering. They are characterized by the occurrence of autoantibodies against structural proteins of the skin. In pemphigus diseases the blistering is intraepithelial, in other bullous autoimmune dermatoses, e.g. the diseases of the pemphigus group, subepithelial.

Classification
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Clinical picture
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The clinical suspicion of the presence of a bullous autoimmune dermatosis is based on the occurrence of usually painful blisters or erosions of the skin (and mucous membranes), which have a slight or very delayed healing tendency. In the pemphigus group of diseases, the flaccid blisters (because they are located intraepidermally) are usually no longer detectable; instead, crusty erosions are often impressive. In the most frequent blistering autoimmune dermatosis, the bullous pemphigoid, the diagnostic leading symptom is always a bulging blister, usually on a reddened area.

Direct immunofluorescence
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Indispensable detection of autoantibodies in blistering autoimmune diseases. The DIF is performed on frozen sections of perilesional or even heart-free healthy skin. In pemphigus diseases the blistering is intraepithelial, in other bullous autoimmune dermatoses subepithelial (e.g. pemphigoids). A further differentiation of the antigens in subepidermal cleft formation is possible by means of the salt-split skin examination.

Indirect immunofluorescence
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Detection method for the characterization of circulating autoantibodies (e.g. desmogleins from the protein family of cadherins in pemphigus vulgaris) on a suitable substrate (e.g. monkey esophagus or rat bladder). Overview tables see below Pemphigus and Pemphigoid. The IIF is one of the indispensable routine methods of an immunological laboratory.

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Last updated on: 29.10.2020