Mucous Membrane Pemphigoid: Evidence Based Updates and Practical Clinical Pearls

The presentation discusses mucous membrane pemphigoid (MMP), a complex and heterogeneous group of sub-epithelial autoimmune diseases, primarily affecting mucosal surfaces and resulting in scarring. It highlights the challenges in diagnosis and management due to the varied clinical presentations and the low levels of circulating autoantibodies often found in patients. The clinical manifestations frequently include oral lesions, particularly gingivitis, with potential involvement of the eyes and other mucosal sites. Due to its diverse nature, MMP requires careful monitoring for disease progression across various sites. The pathophysiology is not fully understood, but involves multiple autoantibodies against specific antigens, including BP180, and is associated with inflammatory responses and scarring mechanisms. Diagnostic approaches include histopathology and direct immunofluorescence (DIF), which can be challenging, and may require multiple biopsies to enhance accuracy. Treatment strategies vary between mild and high-risk cases, often starting with medications like dapsone or mycophenolate for less severe conditions, escalating to therapies such as rituximab or intravenous immunoglobulins for high-risk cases. Emerging treatments including JAK inhibitors, bortezomib, and dupilumab are also noted. The importance of recognizing MMP's heterogeneous nature, ongoing monitoring for mucosal involvement, and repeatedly conducting diagnostic tests to confirm autoimmune activity is emphasized for effective patient management.