Pemphigus vulgaris (PV) is rare autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and most severe form of pemphigus, occurring usually between 40 and 60 years of age. PV is more prevalent among women. Lesions are usually flaccid blisters that are break easily and form painful erosions on the oral mucosa or the skin, which can be disseminated. Almost all patients present mucosal lesions (especially oral mucosa in 90% cases). The most affected areas are the buccal and palatine mucosa, lips, and gingivae. Beside oral mucosal, PV may be involved, including the conjunctiva, nasal mucosa, pharynx, larynx, esophagus, vagina, penis, and anus. The rare manifestations of PV are isolated crusted plaque on face and scalp, foot ulces, dyshidrotic eczema, macroglossia, nail dystrophy, paronychia, and subungual hematomas.
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Oral mucose manifestation
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Skin manifestation
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Nail manifestation
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The treatments for PV are systemic corticosteroids and immunosuppressive drugs. Its mortality is 10% and the main cause of death is septicaemia.
Reference
Porro, A. M., 2019,'Pemphigus vulgaris', An Bras Dermatol, vol. 94, no. 3, pp. 264-278.
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