A middle age person reported flaccid bullae that began in the oropharynx and then spread to the skin. Since the bullae rupture easily, this image shows only erosions. The lesions are painful and there is a fear of secondary infection.Challenge: What's the disease? What's the cause? What's the treatment?
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Blister
This is pemphigus vulgaris which along with bullous pemphigoid are autoimmune blistering diseases. Pemphigus is a group of rare, chronic vesiculobullous diseases of the mucous membranes and skin. Pathogenic autoantibodies in pemphigus vulgaris involve IgG against desmoglein adhesion molecule. First Aid mentions these are anti-epithelial cell antibodies. All patients have oropharyngeal disease. Histology shows intraepithelial acantholysis without disruption of the basement membrane. Treatment is corticosteroids.
Sources: UpToDate; Medline (nlm.nih.gov); First Aid for the USMLE Step 1.
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