A common patient complaint that may be encountered in practice is desquamation of oral tissue. Moderate-to-severe, generalized oral desquamation associated with ulceration and erythema could be associated with systemic mucocutaneous disease. Systemic mucocutaneous diseases that affect the oral cavity include lichen planus, pemphigus and mucous membrane pemphigoid. This article will review the etiology, clinical presentation and management of mucous membrane pemphigoid.