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Clinical Problem Solving: Pathology
February 2003

Pathology Quiz Case 2—Diagnosis

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Copyright 2003 American Medical Association. All Rights Reserved. Applicable FARS/DFARS Restrictions Apply to Government Use.2003

Arch Otolaryngol Head Neck Surg. 2003;129(2):256-257. doi:10.1001/archotol.129.2.254

Pemphigus, which is a rare mucocutaneous disease of immunologic pathogenesis, affects approximately 0.0001% to 0.0005% of the population per year, with a predilection for Ashkenazi Jews.1 Although there are several types of pemphigus (erythematosus, foliaceus, vegetans, vulgaris, and paraneoplastic), PV is the most common form, accounting for 80% of all cases of pemphigus. It often presents in the fifth to sixth decades of life, with an average age at onset of 55 years. There is also a slight female predilection. Pemphigus vulgaris is associated with expression of the human leukocyte antigens DR4, with DQ8 haplotypes in Jewish patients, and DR6, with DQ5 haplotypes in non-Jewish patients.1

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