Pemphigus and mucous membrane pemphigoid: An update from diagnosis to therapy

Publication date: Available online 7 February 2019

Source: Autoimmunity Reviews

Author(s): Alessio Buonavoglia, Patrizia Leone, Rosanna Dammacco, Giuseppe Di Lernia, Massimo Petruzzi, Domenico Bonamonte, Angelo Vacca, Vito Racanelli, Franco Dammacco

Abstract

Pemphigus diseases (PDs) and mucous membrane pemphigoid (MMP) are a group of immune-mediated mucocutaneous disorders clinically characterized by the formation of blisters, erosions and ulcers. The skin and mucous membranes are predominantly affected, with the oropharyngeal mucosa as the initially involved site. Ocular involvement is also a frequent feature of these diseases. Because of the considerable overlap in their clinical presentations, the diagnosis of PDs vs. MMP can be challenging. A recognition of their specific immunological and histopathologic features is crucial in the differential diagnosis. Treatment modalities include systemically administered corticosteroids, steroid-sparing immunosuppressive agents, and biologic therapies (rituximab, intravenous immunoglobulins, and anti-tumor necrosis factor agents). Topical, oral, conjunctival, or intralesional corticosteroids as well as anti-inflammatory drugs and antibiotics are prescribed as needed.

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