Journal
AUTOIMMUNITY REVIEWS
Volume 18, Issue 4, Pages 349-358Publisher
ELSEVIER
DOI: 10.1016/j.autrev.2019.02.005
Keywords
Autoimmune blistering diseases; Cicatricial pemphigoid; Corticosteroids; Desmoglein-1; Desmoglein-3; Immunosuppressive drugs; Pemphigus vulgaris
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Funding
- Italian Association for Cancer Research (AIRC) [20441]
- Fondo di Sviluppo e Coesione 2007-2013 - APQ Ricerca Regione Puglia Programma regionale a sostegno della specializzazione intelligente e della sostenibilita sociale ed ambientale - FutureInResearch
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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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