Pemphigus and pemphigoid are autoimmune diseases that produce blistering of skin and/or mucosa. In pemphigus, intraepithelial blistering is caused by destruction of desmogleins that connect epithelial cells. Its most common variant, pemphigus vulgaris, begins with ulcerating lesions in the mouth, as the outer epithelium of the blister sloughs. Later, skin is also involved. In pemphigoid, the basement membrane is attacked, resulting in subepithelial blisters. Mucosal involvement nearly always begins in the mouth and can spread as far caudal as the larynx. It does not involve the subglottis or trachea (7). Pemphigoid is a subepithelial blistering disease that affects skin and mucous membranes, including the nose and larynx (8).
Diagnosis is established by immunofluorescence to detect the antibodies causing the lesions. Biopsy is often negative because it shows nonspecific necrosis, particularly in the center of ulcerated lesions. Serology is sometimes helpful. Before the advent of drug treatment, pemphigus vulgaris was fatal in 99% of cases, but with treatment including dapsone, steroids, and azathioprine, mortality is 5% to 15%. The mucosal lesions of pemphigus and pemphigoid generally respond well to medical management, but untreated lesions may become infected and cause scarring sufficient to obstruct the airway.
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