Bullous pemphigoid: from bench to bedside.
Bullous pemphigoid (BP) is a chronic, autoimmune, blistering disease observed primarily in the elderly population. Several clinical variants have been described, including classic (bullous), localised, nodular, vegetating, erythrodermic, erosive, childhood and drug-induced forms. Autoantibodies target the BP230 and BP180 antigens, located in the hemidesmosomal complex of the skin basement membrane zone. Subsequent complement activation recruits chemical and cellular immune mediators to the skin, ultimately resulting in blister formation. Both autoantibodies and complement may be detected by various immunofluorescent, immune electron microscopy and molecular biology techniques. Recent trials suggest that potent topical corticosteroids should be considered as first-line therapy. Tetracycline with or without nicotinamide may benefit a subset of patients with mild BP. Oral corticosteroids should rarely exceed 0.75 mg/kg/day and corticosteroid-sparing agents may be useful for recalcitrant disease.
Walsh
Scott R A
SR
Division of Dermatology, University of Toronto, Toronto, Ontario, Canada.
Hogg
David
D
Mydlarski
P Régine
PR
D000287:Administration, Topical; D000900:Anti-Bacterial Agents / Q000008:administration & dosage / Q000627:therapeutic use; D005938:Glucocorticoids / Q000008:administration & dosage / Q000627:therapeutic use*; D006801:Humans; D010391:Pemphigoid, Bullous / Q000139:chemically induced / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology / Q000473:pathology; D013752:Tetracycline / Q000008:administration & dosage / Q000627:therapeutic use
D000900:Anti-Bacterial Agents; D005938:Glucocorticoids; D013752:Tetracycline