Bullous pemphigoid : From bench to bedside

• Published in: Drugs (New York, N.Y.) N.Y.), 2005-01, Vol.65 (7), p.905-926
• Main Authors: WALSH, Scott R. A, HOGG, David, REGINE MYDLARSKI, P
• Format: Article
• Language: English
• Subjects: Administration, Topical; Anti-Bacterial Agents - administration & dosage; Anti-Bacterial Agents - therapeutic use; Biological and medical sciences; Bullous diseases of the skin; Dermatology; Glucocorticoids - administration & dosage; Glucocorticoids - therapeutic use; Humans; Medical sciences; Pemphigoid, Bullous - chemically induced; Pemphigoid, Bullous - complications; Pemphigoid, Bullous - diagnosis; Pemphigoid, Bullous - drug therapy; Pemphigoid, Bullous - epidemiology; Pemphigoid, Bullous - pathology; Tetracycline - administration & dosage; Tetracycline - therapeutic use
• ISSN: 0012-6667; 1179-1950
• DOI: 10.2165/00003495-200565070-00002

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.