Etiology • Autoimmune; trigger unknown • Autoantibodies directed against basement membrane zone
antigens Clinical Presentation
• Vesicles and bullae (short lived) followed by ulceration • Multiple intraoral sites (occasionally gingiva only) • Usually in older adults • 2:1 female predilection • Ocular lesions noted in one-third of cases • Proclivity for scarring in ocular, laryngeal, nasopharyngeal,
and oropharyngeal tissues Microscopic Findings
• Subepithelial cleft formation • Linear pattern IgG and complement 3 (C3) along basement
antigens Clinical Presentation
• Vesicles and bullae (short lived) followed by ulceration • Multiple intraoral sites (occasionally gingiva only) • Usually in older adults • 2:1 female predilection • Ocular lesions noted in one-third of cases • Proclivity for scarring in ocular, laryngeal, nasopharyngeal,
and oropharyngeal tissues Microscopic Findings
• Subepithelial cleft formation • Linear pattern IgG and complement 3 (C3) along basement
membrane zone; less commonly IgA • Direct immunofluorescence examination positive in 80% of cases • Indirect immunofluorescence examination usually negative • Immunoreactants deposited in lamina lucida in most patients
Diagnosis • Biopsy • Direct immunofluorescent examination
Differential Diagnosis • Pemphigus vulgaris • Erythema multiforme • Erosive lichen planus • Lupus erythematosus • Epidermolysis bullosa acquisita
Treatment • Topical corticosteroids • Systemic prednisone, azathioprine, or cyclophosphamide • Tetracycline/niacinamide • Dapsone • See “Therapeutics” section for details.
Prognosis • Morbidity related to mucosal scarring (oropharyngeal,
nasopharyngeal, laryngeal, ocular, genital) • Management often difficult due to variable response to
corticosteroids • Management often requires multiple specialists working in
concert (dental, dermatology, ophthalmology, otolaryngology)
nasopharyngeal, laryngeal, ocular, genital) • Management often difficult due to variable response to
corticosteroids • Management often requires multiple specialists working in
concert (dental, dermatology, ophthalmology, otolaryngology)