Pathology

Mucocutaneous zone

Antibody

Dermatology

Immunology

Cicatricial pemphigoid

Pemphigoid

Disease

Bullous pemphigoid

Mucous membrane

Rituximab

Pemphigus vulgaris

Pemphigus

Medicine

Autoimmunity Reviews

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.

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