A 29 y.o. woman presents with painful labial ulcers
What is your differential ?
Our patient has ruled out for syphilis, herpes, EBV, and HIV which are the most common causes of labial ulcers. . She has aphthous ulcers in her mouth as well as on the labia which is why the diagnosis of Behcet’s is considered likely. She was treated with steriods and improved but as they were tapered her symptoms recurred and she required repeat admissions for labial ulcers.
The diagnosis of Behcet’s is being considered but is difficult to make. It involves inflammation of the blood vessels and is thought to be an autoimmune vasculitis.The usual rheumatological workup is often negative as in our patient with negative. ANA, RF, PR3 antibody, MPO antibody, ANCA, SSA SSB, Smith antibody, SCL-70 and anti-Jo-1.
Behcet’s disease was first described in 1937 by Dr. Hulusi Behcet , a dermatologist from Turkey. He described it as a triple symptom complex: aphthous stomatitis, genital ulcers and relapsing uveitis. Behcet’s is more prevalent around the ancient “Silk Road” with high prevalence rates in Turkey, Iraq, Iran and China. The disease is strongly associated with the presence of the HLA-B151 allele with 81% of Asian patients with the disease expressing the allele. In the US only 13% possess the allele.
The Silk road was a system of ancient trade routes established during the Han Dynasty in 130 BCE; transporting silk, porcelain, gold and spices from Asia to Europe. In 1453 the Ottoman empire closed the routes and refused to trade with the West.
CRITERIA FOR DIAGNOSING BEHCET’S
The criteria for diagnosing Behcet’s include recurrent oral aphthae plus two of the following:
Genital ulcers- are most commonly caused by STIs. With herpes and syphilis being most common. Less common are lyphogranuloma venereum, donovanosis and chancroid
Eye inflammation- only 25% of the patients in the US develop eye involvement
Skin lesions- papulopustular lesions resembling acne can be found
Pathergy- A pathergy test involves pricking the skin with a needle to see if a red spot appears in the next day or two; people with Behcet’s often have a positive pathergy test although only 10% of the people with the disease in the US have pathergy.
Behcet’s can be confused with sarcoidosis and Crohn’s disease. They both can present with uveitis, arthritis, erythema nodosum and oral ulcers. Reiter’s disease is also similar but the urethritis found in Reiter’s does not appear in Crohn’s or sarcoid. Intestinal involvement is considered rare in the Middle East in patients with Behcet’s but is more common in the US.
A patient with perforated bowel from Behcet’s disease. ASCA(anti-saccharomyces cerevisiae antibodies) are seen in 45% of patients with bowel involvement in Behcet’s
The treatment of Behcet’s is immune suppression. Generally steroids are the first line but other immunosuppressives are being studied. A trial of secukinumab is currently being studied in a clinical trial.
Kilian N, SawalhaA. Behcet’s disease in the United States: A congle center descriptive and comparative study. European Journal of Rheumatology. 2017 dec;4(4):239-244.
Ahmed Z, Rossi M, Yong S, et al. Behcet’s disease departs the Silk Road: a cas report and brief review of literature with geographical comparison. J Community Hosp Intern Med Perspect 2016;6:30362.
Cakir N, Dervis E, Benian O, et al. Prevalence of Behcet’s disease in rural western Turkey: a preliminary report. Clin exp Rheumatol 2004:22(4 Suppl. 34):53-5.