Pemphigus vulgaris and paraneoplastic pemphigus are 2 subtypes of pemphigus that involve the oral mucosa. These autoimmune blistering disorders have antibodies targeted against proteins of keratinocyte adhesion, thereby causing acantholysis. Clinical findings include oral erosions and flaccid cutaneous bullae and erosions. Further malignancy workup in patients with suspected paraneoplastic pemphigus is warranted. Retrospective uncontrolled studies suggest that immunosuppressive agents reduce mortality in pemphigus vulgaris and cohort uncontrolled studies of rituximab, a monoclonal antibody against CD20, suggest it is an effective treatment for refractory patients. Ongoing studies will define its role in early disease.
Key points
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Pemphigus vulgaris and paraneoplastic pemphigus manifest as flaccid mucosal bullae leading to erosions.
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Mucosal biopsy and direct immunofluorescence are necessary to differentiate pemphigus vulgaris and paraneoplastic pemphigus from other mucosal diseases. Indirect immunofluorescence and enzyme linked immunosorbent assays can aid in the diagnosis and be may be useful to monitor disease activity.
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Paraneoplastic pemphigus is normally associated with an underlying malignancy, usually of lymphoid origin. A complete blood count, computed tomography scan of the chest, abdomen, and pelvis, and a bone marrow biopsy may be indicated.
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Immunosuppressive agents can be used as steroid-sparing agents.
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Rituximab, an anti-CD20 monoclonal antibody, helps in treatment-refractory pemphigus vulgaris. Defining the role of rituximab in earlier disease, and well as its safety profile, will require larger prospective studies.
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