Recurrent Oral Ulcers in a Refugee
Am Fam Physician. 2018 Mar 15;97(6):411-412.
A 41-year-old refugee from Africa presented with seven months of recurrent painful oral ulcers. The ulcers were present prior to his arrival in the United States. The ulcers initially responded to oral corticosteroids but recurred one month after treatment. Associated symptoms included erythema, bleeding, dysphagia, odynophagia, anorexia, abdominal pain, and rectal bleeding. He had no fever or night sweats. He was treated for conjunctivitis and a nonpainful penile ulcer two weeks before presentation. His medical history was significant for hepatitis B with cirrhosis, treated syphilis, latent tuberculosis, Candida esophagitis, schistosomiasis, and rectal bleeding.
The patient was afebrile on physical examination. He had erythema and ulcers that involved the lips, tongue, and oropharynx with tender and friable mucosa (Figure 1). Immunofluorescence with enzyme-linked immunoreceptor assay was positive for desmoglein 1 and 3 antibodies.
Based on the patient's history and physical examination findings, which one of the following is the most likely diagnosis?
A. Behçet disease.
B. Erythema multiforme.
C. Pemphigus vulgaris.
D. Recurrent aphthous stomatitis.
The answer is C: pemphigus vulgaris. This rare and life-threatening blistering disease is caused by circulating autoantibodies against desmosomes. This results in cell-cell detachment (acantholysis) and development of intraepithelial blisters.1,2 The incidence of pemphigus vulgaris is approximately 0.1 to 0.5 per 1 million persons per year, with a higher incidence in individuals of Indian, Malaysian, Chinese, Japanese, Middle Eastern, or Jewish descent.3 It affects men and women equally, with an average age of onset between the fourth and sixth decades of life.3
Oral mucosal erosion is the most common initial manifestation of pemphigus vulgaris, occuring in 80% of patients with the disease. It can also involve the eyes and genital area, causing conjunctivitis and genital ulcers.3 Diagnosis is confirmed with direct immunofluorescence microscopy demonstrating autoantibodies against desmoglein 1 and 3. Serum
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1. Mustafa MB, Porter SR, Smoller BR, Sitaru C. Oral mucosal manifestations of autoimmune skin diseases. Autoimmun Rev. 2015;14(10):930–951....
2. Ruocco V, Ruocco E, Lo Schiavo A, Brunetti G, Guerrera LP, Wolf R. Pemphigus: etiology, pathogenesis, and inducing or triggering factors: facts and controversies. Clin Dermatol. 2013;31(4):374–381.
3. Kim J, Hertl M, Korman NJ, Murrell DF. Pemphigus vulgaris. In: Blistering Diseases: Clinical Features, Pathogenesis, Treatment. Berlin, Germany: Springer; 2015:283–288.
4. Ruocco E, Wolf R, Ruocco V, Brunetti G, Romano F, Lo Schiavo A. Pemphigus: associations and management guidelines: facts and controversies. Clin Dermatol. 2013;31(4):382–390.
5. Rokutanda R, Kishimoto M, Okada M. Update on the diagnosis and management of Behçet's disease. Open Access Rheumatol. 2015;7:1–8.
6. Preeti L, Magesh K, Rajkumar K, Karthik R. Recurrent aphthous stomatitis. J Oral Maxillofac Pathol. 2011;15(3):252–256.
This series is coordinated by John E. Delzell Jr., MD, MSPH, Associate Medical Editor.
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