Behcet’s Syndrome: Causes, Symptoms, Diagnosis and Treatment

Behcet’s syndrome is a multisystem disorder which is characterized by recurrent painful ulcer in mouth, genital tract, inflammation in eyes, skin eruptions, gastrointestinal, neurologic, pulmonary and renal manifestations. This disease is chronic and runs a prolonged course with intermittent remission and relapse. Different areas of the body are affected at each time.

The exact cause is not known but the main pathology is understood to be inflammation of blood vessels throughout the body. Behcet’s syndrome is believed to develop as an autoimmune bodily response environmental and genetic factors. The disease manifestations are predominant between 20 and 30 years of life. Both males and females are equally affected. There is symptomatic besides use of steroids and cytotoxic drugs. Blindness and CNS involvement are frequent complications.

Causes:

Behcet’s syndrome is named after a Turkish professor of dermatology who described the clinical symptoms of this condition in detail. The exact cause of Behcet’s syndrome is not clear. The disease is rare and it is common in Mediterranean countries, Japan and China.

It is a chronic autoimmune disease with episodes of frequent relapses. In autoimmune disease the immune system of the body attacks its own healthy cells and tissue. Although the exact cause is not found, the underlying pathology is inflammation of the blood vessels (vasculitis) throughout the body. Genetic and environmental factors are suspected for the underlying vasculitis. Researchers believe microbial pathogens (bacteria and virus) are believed to initiate immune dysfunction in people who are genetically susceptible to Behcet’s syndrome.

Symptoms:

The symptoms and sign of Behcet’s syndrome depend on the area of the body affected. Inflammation of arteries and vein can occur in any part of the body. The areas usually affected because of vasculitis in Behcet’s syndrome are eyes, skin, mouth, genitals, joints, vascular system, brain, digestive system, lungs etc. It is a chronic rare disease having frequent episodes of relapses and remission. Over a period time, the symptom severity decreases. Following are some of the signs and symptoms produced by the affected area:

• Mouth: Ulcers in mouth is one of the prominent and earliest manifestations of Behcet’s syndrome. Mouth ulcers are painful, shallow or deep, round or oval, with white or yellow base. They can be large or small in size. Ulcers can be present anywhere in the oral cavity. There can be single or multiple ulcers. The sores generally heal within 20 days without leaving any scars.
• Genital ulcers: Ulcers in genital region of males develop on scrotum more than on shaft or glans of penis. The lesions are deep and heal leaving scars tissue. In women the ulcers are on vulva, vagina, and on cervix. The genital ulcers are painful both in males and females.
• Skin lesions: Skin manifestation occurs in almost 60 percent of patients suffering from Behcet’s syndrome. Red tender nodes present under the skin due to inflammation in the subcutaneous layer below the skin is frequently encountered symptom in Behcet’s syndrome. In some people lesions similar to acne develop on the body. The skin lesions usually are in the lower limbs, face and neck.
• Ocular manifestation: Patient may suffer from inflammation in the eyes. The condition called iritis and uveitis. It produces pain, redness, sensitivity to light, excessive tears and blurry vision in one or both the eyes. In Behcet’s syndrome the eye symptoms frequently come and go.
• Joints: Arthritis (pain, swelling and stiffness of joints) is common in Behcet’s syndrome. It mostly affects knee, elbow, ankle and wrist joints. The symptoms are self limited and recurrent. There is no deformity noted.
• Digestive system: At least 25 percent of patient develops gastrointestinal symptoms in Behcet’s syndrome. The symptoms mostly develop many years after the oral ulcers. Symptoms include reduced appetite, pain in abdomen, diarrhea, bleeding from rectum,
• Brain: Patient may suffer from headache, fever, disorientation, confusion, stroke, memory loss, etc. The symptoms can occur due to aseptic meningitis.

Diagnosis:

There is no specific confirmatory test to diagnose Behcet’s syndrome. Hence diagnosis is ascertained from the clinical picture of the disease. Certain criteria are required for diagnosis of this condition.

• Recurrence of mouth ulcers at least three times in a year.
• Recurrent painful genital ulcers.
• Inflammation in eye diagnosed by an ophthalmologist.
• Skin lesion
• Positive pathergy test: It is a skin test done by a doctor. A small sterile needle is inserted in the forearm skin. The area that has been pricked by the needle is examined after two days. If there is a small red bump present at the site, it indicates positive pathergy test. This means even for a small injury your immune system is extremely oversensitive.

Besides mouth ulcers any other two criteria are required to establish Behcet’s syndrome in a patient.

Treatment:

There is no cure of Behcet’s syndrome. Treatment is focused on alleviating the symptoms and preventing complications. Treatment also depends on the severity of symptoms. In between when the symptoms disappear temporarily patient does not require any treatment. During the flare ups local application of corticosteroids is found to be beneficial. For example in case of skin lesions ointments and creams containing corticosteroid is applied on the lesion to alleviate pain and redness. For mouth ulcers local mouth paints containing corticosteroid is found effective. Alternatively mouth rises containing corticosteroid is used to reduce pain and inflammation. In case of eye affection, eyedrops containing corticosteroids are used.

If local application of medications is not beneficial, doctors may also opt for systemic steroids and other medications that help to suppress immunity.

Proper rest, healthy diet and mild exercise also help to reduce the episodes. Usually patient lives a productive life without any complication if he takes medications as recommended by his doctor.