Immunobullous Diseases

Enno Schmidt, Richard Groves


The immunobullous disorders represent a group of conditions characterized by an antibody‐mediated autoimmune response against structural elements of the skin resulting in blistering of the skin and mucosae. Antibody targets include proteins in hemidesmosomes and the basement membrane zone (pemphigoid group), anchoring fibrils in the papillary dermis (epidermolysis bullosa acquisita), desmosomes (pemphigus group) and epidermal transglutaminase (dermatitis herpetiformis). If left untreated, immunobullous disorders may be associated with significant morbidity and mortality so prompt, accurate diagnosis and treatment are mandatory. Therapeutic options include systemic corticosteroid therapy, steroid‐sparing immunosuppressants (e.g. azathioprine and mycophenolates) and immunomodulating agents (e.g. dapsone and tetracyclines). In severe and/or refractory patients, intravenous immunoglobulin, immunoadsorption and the anti‐B‐cell drug rituximab are applied.
Keywords autoimmunity, autoantibody, blister, bullous, complement, desmosome, hemidesmosome, dermal–epidermal junction, pemphigus, pemphigoid, BP180, BP230, desmoglein, dapsone, rituximab, immunoadsorption


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