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Abstract

Mucous membrane pemphigoid (MMP), an autoimmune subepithelial blistering disease that predominantly affects the mucous membranes, is usually diagnosed in elderly adults. Early diagnosis of MMP is crucial because it tends to run a chronic and progressive course with the potential for devastating scarring of the mucous membranes that may lead to blindness and airway compromise. A subtype of MMP, anti-laminin-332 MMP, is a rare blistering disorder in which autoantibodies are directed against laminin-332 (formerly epiligrin), a structural protein of the epidermal basement membrane. Herein we report what we believe to be the youngest patient diagnosed with anti-laminin-332 MMP, a 9-year-old girl with disease affecting only the oral, pharyngeal, and laryngeal mucosa, with no skin involvement.