Clinical Phenotype of Pemphigus Vulgaris

PV is a relatively rare disorder that primarily affects individuals in the 3rd to 5th decade without gender preference (Huilgol and Black, 1995; Nousari and Anhalt, 1999). Clinically, PV is characterized by flaccid blisters/erosions of the mucous membranes and the skin. In the majority of patients, the oral mucosa is primarily affected but other mucous membranes may be involved as well. Initial blisters rapidly rupture, leading to painful chronic lesions that may affect the larynx and the pharynx in addition to the oral mucosa. Once the disease progresses, skin lesions may occur at any site of the integument, but there is a preferential involvement of the trunk. Due to extensive blistering of skin and mucosa, the prognosis of PV used to be fatal before introduction of glucocorticoids as the major therapeutic strategy. The natural course of the disease is progressive, with death occurring within a few years of onset due to sepsis.

Neonatal pemphigus may occur due to the diaplacentar transfer of anti-Dsg3 IgG4 from mothers with PV to their unborn children. After birth, the newborns exhibit crusty erosions of the skin with the histopathological findings of PV Once circulating autoAbs are degraded, these skin lesions disappear after a few months.

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