Published Paper Details:

An autoimmune response against two hemidesmosomal proteins in the dermal-epidermal junction, BP180 and BP230, is the characteristic of bullous pemphigoid (BP), a blistering dermatosis. BP180 is an extracellular domain-bearing transmembrane glycoprotein, while BP230 is intracellularly bound and associated with the hemidesmosomal plaque. Most BP patients possess autoantibodies that bind to the noncollagenous 16A domain (NC16A) of BP180, an immunodominant region of BP180 that is extracellularly located adjacent to the protein's transmembrane domain. Autoreactive T and B cell responses to BP180 in BP patients have been reported. In mice, a bullous skin disease with a very close analog of human blood pressure is induced by passive antibody transfer to the mouse BP180 ectodomain. In this model of animals, the formation of lesions relies on mast cell degranulation, complement activation, and neutrophil and eosinophil recruitment. Dermal-epidermal separation occurs in cryosections of human skin when autoantibodies against BP180 are co-incubated with leukocytes. Granulocytes secrete proteinases that cause loss of cell-matrix adhesion. The development of new treatment strategies for BP may be facilitated by our increasing knowledge of its pathophysiology.