Epidermolysis Bullosa Acquisita
Epidermolysis bullosa acquisita (EBA) is a form of pemphigoid which may come with scarring that looks like dystrophic epidermolysis bullosa hereditaria. This subtype with scarring is named mechanobullous EBA, because blisters are evoked by sudden mechanical trauma, such as hitting the back of the hand to the edge of a table. The other subtype with erythematous lesions without scarring is named inflammatory EBA and may look like bullous pemphigoid. The mucous membranes can be involved in both subtypes. The pathogenesis is mediated by IgG or IgA against type VII collagen, which is the component of anchoring fibrils below the lamina densa. Diagnosis is confirmed by detecting u-serrated linear pattern of immune depositions with direct immunofluorescence microscopy. The pathogenesis of both clinical subtypes is unknown and is not related to binding of a particular epitope of the autoantigen. EBA is associated with systemic lupus erythematosus and colitis ulcerosa. The disease is relative refractory to treatment.
KeywordsHemidesmosome Immunoglobuline Basement membrane Autoimmune disease Vesiculobullous disease Pemphigoid