Bullous Pemphigoid

Dr. Wendy Levinbook, MD discusses Bullous Pemphigoid. See much more at www.dermnet.com PLEASE RATE AND COMMENT!!! This disorder typically affects patients over 60 years of age, but it can rarely happen in children. There is no racial or ethnic predilection and the incidence is equal in males and females. Clinically, bullous pemphigoid (BP) is characterized by huge tense bullae that are frequently preceded by red, urticarial patches or plaques. Lesions are most generally located on the lower abdomen, inner thighs, and flexor forearms despite the fact that they might happen anywhere. Mucous membrane involvement is uncommon. Lesions are usually associated with marked pruritus but they do not scar. BP is an acquired autoimmune disorder that occurs secondary to the formation of antibodies that interact with bullous pemphigoid antigen 1 (230 kDa intrahemidesmosomal antigen) and bullous pemphigoid antigen 2 (180 kDa hemidesmosomal transmembrane antigen, also identified as type XVII collagen). This interaction is then followed by an inflammatory response leading to dermal-epidermal separation. On electron microscopic exam, blister formation occurs in the lamina lucida, between the basal cell membrane and the lamina densa. On histopathologic exam, lesions show a subepidermal blister with fluid, fibrin, and inflammatory cells which includes several eosinophils. Direct IF of perilesional skin demonstrates linear C3 and IgG. Indirect IF utilizing normal stratified squamous epithelia (such as human epidermis or monkey ...
Video Rating: five / five

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