A 46-year-old Hispanic male referred by his dentist presents for diagnosis and treatment of painful mouth lesions. The lesions were first noticed approximately 2 weeks previously. He denies prior history of fever blisters or canker sores. Social history is positive for moderate alcohol intake and tobacco use. He was recently placed on a thiazide diuretic for control of hypertension. Examination reveals glistening ulcerations of the buccal mucosa and scattered flaccid bullae of his trunk.
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Pemphigus vulgaris is a life-threatening autoimmune disease characterized by multiple blisters. In many cases (70%-90%), the blisters first form on the oral mucosa before spreading to involve the skin. Pemphigus vulgaris is induced by autoantibodies against keratinocyte cell surface components. It is most common in people in their 40s and 50s, with an equal male-to-female ratio.1,2
Oral lesions often form within areas of higher friction or trauma (cheek, tongue, lower lip) but can be found on any mucocutaneous surface. The blisters are thin walled and eventuate in painful erosions that interfere with chewing and swallowing. Diagnosis is made by histopathologic examination and confirmed by direct immunofluorescence.2,3
Traditional treatment of pemphigus vulgaris consists of high-dose systemic corticosteroids with steroid-sparing immunosuppressants (ie, azathioprine and mycophenolate mofetil) added as prednisone is slowly tapered.4 Rituximab (approved in 2018 as therapy for pemphigus) has enhanced treatment, allowing rapid clearing and use of lowered doses of prednisone at onset.5
Disclosures: Kevin Anderson is a physician assistant student at Arcadia University.
Dr Schleicher is director of the DermDox Center for Dermatology, as well as an associate professor of medicine at Commonwealth Medical College and a clinical instructor of dermatology at Arcadia University and Kings College.
1. Rajendran R. Diseases of the skin. In: Rajendran R, Sivapathasundharam B, eds. Shafer’s Textbook of Oral Pathology. 6th ed. Noida, India: Elsevier; 2009: 797-843.
2. Fassmann A, Dvořákován N, Izakovicova Holla L, Vanĕk J, Wotke J. Manifestation of pemphigus vulgaris in the orofacial region. Case report. Scripta Medica (brno). 2003;76: 55-62.
3. Shamim T, Varghese VI, Shameena PM, Sudha S. Pemphigus vulgaris in oral cavity: clinical analysis of 71 cases. Med Oral Patol Oral Cir Bucal. 2008;13(10):E622-626.
4. Strowd LC, Taylor SL, Jorizzo JL, Namazi MR. Therapeutic ladder for pemphigus vulgaris: emphasis on achieving complete remission. J Am Acad Dermatol. 2011;64:490-494.
5. Murrell DF, Sprecher E. Rituximab and short-course prednisone as the new gold standard for new-onset pemphigus vulgaris and pemphigus foliaceus. Br J Dermatol. 2017;177(5):1143-1144.