Recurrent Ulcers as a Sign of Behçet Disease

Authors:
Andrew Gross, BA; Laura Rosas, BBA; Denise Woody-Gross, MD; and Ankur Kamdar, MD
McGovern Medical School at the University of Texas Health Science Center at Houston

A 15-year-old girl presented for a well-child visit. During the review of systems, she stated that she had had vaginal pain on and off since 8 years of age.

History. She had mentioned the pain to her mother but had attributed it to the swimming lessons she had been taking at the time. At age 14, as she inserted a tampon using a mirror, she had noted pain upon insertion and ulcers near her vagina. The lesions waxed and waned over time but never resolved.

Upon further questioning, she also reported having recurrent oral ulcers, which had begun at age 6 and had occurred 2 to 3 times a month, with each occurrence lasting 7 to 10 days. The longest period without oral ulcers had been approximately a week. She had made a few emergency department visits for the oral ulcers but had been told that they were stress-related and had been treated with topical analgesics. At her gynecologic visit, she had mentioned the genital ulcers but not the oral ones.

The girl denied any history of sexual contact. She also denied any history of fever, weight loss, dry or red eyes or visual disturbances, diarrhea, or joint pain.

Physical examination. Physical examination findings were notable for the presence of ulcers on the oral mucosa (Figure 1) and on the genital mucosa near the vaginal introitus (Figure 2).

Diagnostic tests. Given that the genitourinary tract lesions could have been infectious or inflammatory, laboratory studies were ordered, and she was referred to a rheumatologist for further assistance. Test results for herpes simplex virus (HSV) types 1 and 2, HIV, and syphilis were negative. Results of further laboratory evaluations were negative, including inflammatory markers, rheumatoid factor, antinuclear antibodies, anti–double-stranded DNA antibodies, anti-SSA and anti-SSB antibodies, and anti-Smith and anti-RNP antibodies.

The differential diagnosis of oral and vaginal ulcers when considered separately is extensive. However, when considered together, the list includes infectious etiologies such as HSV and syphilis, and an inflammatory process such as Behçet disease (BD). Although herpetic infections can be recurrent, subsequent infections are infrequent and less severe. With syphilis, the primary chancre is painless, but the mucosal ulcers of secondary syphilis are painful. When our patient’s test results for HSV and syphilis were negative, BD was suspected.

Discussion. BD is a chronic relapsing inflammatory disease characterized by a triad of oral and genital lesions and uveitis. It was first described in the 1930s by Turkish dermatologist Hulusi Behçet.^1,2 The diagnosis is based on the International Criteria for Behçet’s Disease.^3,4 Other typical manifestations include arthritis, uveitis, dermatitis, and pathergy (cutaneous hyperreactivity in response to minor cutaneous trauma). More severe manifestations involve the central nervous, pulmonary, gastrointestinal, cardiovascular, and musculoskeletal systems.^5,6

Recurrent oral ulcers typically are the earliest manifestation of BD. While aphthous ulcers occur in 20% of the general population, all persons with BD present with oral ulcers, and more than 80% have it as their initial lesion.^5-7 Genital ulcers typically present in 5% to 93% of patients with BD and are the initial lesion in up to 20% of them. The genital ulcers are painful and more irregularly shaped than the oral ulcers. In women, they can affect the vulva, the vagina, and occasionally the cervix, and they may create a fistula with the urinary tract.^5-7

Although the etiology of BD is unknown, infectious, autoimmune, and genetic causes are suspected. Worldwide, it presents in approximately 1 in 10,000 persons; the highest prevalence—more than 400 in 10,000 persons—occurs in Turkey.⁸ BD is associated with the HLA-B51 haplotype and usually presents between 20 and 30 years of age.⁸ It is rare before puberty, but when it occurs in children, they have a lower prevalence of genital ulcers and a higher prevalence of ocular manifestations.⁸

Treatment is aimed at reducing symptoms and preventing chronic damage and is highly dependent on the site and severity of symptoms. Anti-inflammatory agents (ie, corticosteroids) and immunosuppressant drugs have been used successfully.⁵

Because patients may incorrectly categorize their symptoms to certain systems, they may not mention them to a seemingly unrelated specialist; therefore, a thorough review of systems is needed at all well-child visits. When adolescents present with recurrent ulcers, consider further exploration and workup and consider BD as part of the differential diagnosis.

Outcome of the case. The patient met the criteria for a diagnosis of BD, and she was started on prednisone therapy for the recurrent oral and genitourinary tract ulcers. 

References:

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