Pathogenesis of Bullous Pemphigoid

Author(s): Cut Putri Hazlianda* and Widya Gabriella Manurung


The most prevalent autoimmune blistering illness of the mucous membranes and skin is Bullous Pemphigoid (BP). BP usually has the general appearance of tense skin blisters and pruritus. The disease is mediated by autoantibodies to the hemi-desmosomal proteins BP180 and BP230, which lead to blister formation. Inflammatory cells including eosinophils, monocytes, lymphocytes, neutrophils, and macrophages are present in the bullous cavities and upper dermis. Several proteinases, including Matrix Metalloproteinase (MMP)9, collagenase, plasmin, neutrophil elastase, are found in BP blister fluid, which have an important duty in the formation of sub-epidermal blister by degrading Extracellular Matrix (ECM) proteins. Eosinophils, Substance P (SP) and their receptors (NK1R, OSMRβ, and IL-31RA), IL-13, basophils, and periostin have the potential to cause itching in BP.

image 10.4303/JDAR/236275

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