By Maria Razzaq, Farooq Ali Khan, Abhishek Kumar, Raamesh Gowri Raghavan, and Sukant Khurana*
What if the mouth ulcer you ignored was not an ordinary but a symptom of something worse? Mouth ulcers and Uveitis are the most common early manifestations of Behçet Disease.
An inflammation of the blood vessels, Behçet Disease was first described by a Turkish doctor and scientist, Hulusi Behçet (1937) after observing them in a patient in 1924.
§ Classification — It can be classified as a mucocutaneous and arthritic disease.
§ Symptoms — It is characterized by a triple symptom disorder including recurrent apthous ulcers, genital sores and uveitis.
o Mouth — The symptoms can range from sores to ulcers.
o Skin — It involves acne -like sores or tender raised nodules on the epithelial surface.
o Genitals — Red open sores on the scrotum or the vulva.
o Eyes — Inflammation of the Uvea (Uveitis).
o Other symptoms include Joint swelling in knees and pain; Diarrhea, abdominal inflammation.
o Brain and Nervous System — It can vary from headaches, fever to disorientation, memory loss and hemorrhages.
§ Etiology –
No exact cause has been yet described; it is considered as an autoimmune disorder which can be triggered by various environmental factors or/and virus, bacteria (Streptococcus) etc. It can also be caused by immune complexes that lead to the inflammation of small and medium sized blood vessels and strong association with certain HLA type. Some scholars suggest the cause to be the inflammation of epithelial cells caused by immuno -competent T-lymphocytes and plasma cells. Several genetic and environmental factors can also be associated with its development.
§ Epidemiology –
More cases have been found in Middle east and far eastern countries like Turkey, Japan, China etc. It’s occurrence is found to be highest along the old silk route from Middle east to china.
§ Risk Factors –
o It has been diagnosed mainly in the people in their 20s and 30s
o This condition is more severe in males.
o In Germany, Japan, and Brazil, the disease is slightly more common in females. Behçet disease is more common in females with the ratio of 5:1 in the United States of America.
o Certain people carry the risk factors in their genes.
§ Complications –
o Severe uveitis can lead to blindness.
o Neurological complications can cause memory deficits and loss of brain function.
o Pulmonary and arterial aneurysms coupled with Behçet disease, if ruptured have a high mortality rate.
o Vasculitis and Thrombosis are one of the major causes leading to the death of an individual.
§ Diagnosis –
o Mouth sores — have to be recurrent (at least thrice in a year)
o Genital sores
o Eye inflammation
o Skin Sores
o Positive Pathergy Test
o Any two symptoms apart from mouth sores conclude the diagnosis of Behçet disease.
§ Misdiagnosis –
o Mouth sores can however be easily mistaken for Canker Sores but they differ in a great deal. Canker Sores are less than 1mm in diameter and heal without scarring however this is not the case in these ulcers which are apthous in nature and larger as well as heal with scarring.
o These sores can also be mistaken for herpetic sores; however they are quite different in appearance — They occur singly while that of herpes are found in clusters.
§ Cure –
o Since it’s an autoimmune disorder, no exact cure exists; only symptomatic treatment can be given.
o In early stages, Corticosteroids containing skin creams, gel, ointments, mouth rinses, eye drops are given to treat the sores and inflammation. Side effects include weight gain, persistent heartburn, high blood pressure and osteoporosis.
o Colchicine (Colcrys) is said to improve the symptoms with arthritis. Side effects are lower sperm count which may lead to male infertility.
o Immunosuppressants like Azathioprine, Cyclosporine, Cyclophosphamide are also given to control the symptoms. Side effects include increased risk of infection, high blood pressure, low blood counts.
o Interferon alfa 2b (Intron 2A) when used alone or with drugs is said to control skin sores, joint pain, eye inflammation. Side effects include flu -like symptoms.
o Infliximab (Remicade) works well against TNF — Tumor Necrosis Factor. Side effects include headache, skin rash and increased chances upper respiratory infection.
o In extreme cases, Plasmapheresis is used in cases of emergency.
§ Recent Findings –
o In a recent case study by Emmunqil H, Yasar Bilqe et al, it was concluded that intracardiac thrombus was a rare albeit serious complication of Behçet diease. Cases from four Neurology centers were studied that included Ankara University, Ege University, Hacettepe University and Eskişehir Osmangazi University Hospitals. Out of the candidates selected, 22 of them were diagnosed with intracardiac thrombus and the mean age presented out to be 29 years. Initial symptoms included chest pain, dyspnea, pulmonary arteritis, fever and haemoptysis. The medication given included Prednisone, Cyclophosphamide, Warfarin and Azathioprine.In more severe cases, it was combined with interferon 2b treatment.
It was thus concluded that Intracardiac Thrombus was severe complication that usually presented itself in males with predominance of occurring on the right side of the heart. However it was found to be treatable with early aggressive immunosuppressant and/or anti -coagulant therapy. Only one candidate died due to severe complications.
o According to a case report presented by Takayama K et al wherein a young pregnant woman of 30 presented with the symptoms of early manifestations of the disease at 12 weeks into pregnancy. After a treatment of Infliximab, the symptoms improved and she successfully gave birth to a 3.9 kg baby without any abnormality at 39 weeks. The symptoms reappeared and were thus controlled by a dosage of Infliximab. It was thus concluded that Infliximab may be safe and effective for Behçet’s Disease during pregancy.
§ Support Groups and Organizations Worldwide –
American Behçet Disease Association (ABDA): Presently situated in Rochester, Michigan, ABDA was founded in 1970 by Sheila Gregory, mother of a young Behçet’s patient in Orange County, California. Formerly it was known as American Behçet Disease Foundation. The motto of this foundation is ‘ Support — Educate — Advocate — Research.’ The organization can be reached at its volunteer staffed hotline 1–800–7BEHÇET (1–800–723–4238) wherein patients are trained to answer the calls.
UK Behçet’s Syndrome Society: Founded by Judith Buckle in 1983, the society aims at not only providing care and support to the patients suffering from Behçet’s disease but also provide financial aid, foster education, promote research and culminate interest in the medical organizations regarding this disease and collaborate with them.
Behçet’s International: It is an international organization that aims collaborating with all the international Behçet’s support group and forming a network thus ultimately assisting them achieve effective support.
§ Some of the other names by which Behçet disease is known:
o Adamantiades-Behçet disease
o Behçet syndrome
o Behçet triple symptom complex
o Behçet’s syndrome
o Old Silk Route disease
o Triple symptom complex
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