Oral Ulcers (Aphthae)

Oral ulcerations are encountered in almost every Behçet patients. However it is known that 1-3% of patients manifest other signs of the disease but not oral ulcerations. These lesions are mostly the first signs of the disease. Patients with only oral lesions for years are not rare. Although in most of the times the oral lesions in Behçet’s syndrome can not be differentiated from the common recurrent oral ulcers, there are some differences such as multiplicity in number and higher frequency of recurrences. Aphthous lesions in Behçet’s disease recur with a frequency of once or twice a month and heals in few days to one week. The nourishment of the patient may be impaired due to the fact that the oral lesions are several in number and sometimes painful.

Genital Ulcers

Genital lesions appear as small red elevated acne-like lesions and evolve to a slow healing punched-out-like lesions. They mostly heal with a residual scar. In the figure on the left, a post lesional scar is shown. Compared to the oral lesions, genital ulcers are fewer in number, and it takes a longer time for them to heal.

   In Behçet’s syndrome, similar lesions can also be observed in other body parts besides the genital area. These lesions can appear after the ulceration of the acne like lesions in skin folder such as the armpits and the groin.

Skin Manifestations

   The skin manifestations of Behçet’s syndrome can be grouped into three:
(i) red, painful swellings;
(ii) acne-like lesions;
(iii) lesions associated with the diseases of skin vessels.

* Nodular Manifestations

   These are tender, painful, round or oval lesions that appear spontaneously in the dimensions of about a filbert to a walnut . They sometimes can form a linear pattern as if following the trace of a vein. They are especially seen in the legs. They heal within 10-15 days without ulcerating, sometimes leaving a residual pigmentation.

* Acne-like lesions

They are seen as purulent acne without the microorganisms. The most commonly involved areas are neck, back, face, chest, arms and legs, hips, groins and genital area. They are seen in 60-85% of cases. Morphologically same with the ordinary acne.

Pathergy (Non-specific skin reaction)

The test is performed by puncturing the forearm skin with sterile needles. For the expected reaction to occur, the needle should penetrate into the skin layer called the dermis. The early reaction, which appears within 24 hours and maximizes in 48 hours, is a 1-2 mm elevated lesions surrounded by a reddish area. The lesions can stay as it is or mostly develops in to a 1-5 mm sterile purulent one. In the figure on the right, such a reaction is demonstrated. The test has a high specificity and sensitivity in Behçet’s syndrome patients in the Turkish population. Despite the high positivity of about 50-80% in Turkey, Japan and the other Mediterranean countries, positivity is seldom the cases in Britain and America. The reaction is more pronounced in males compared to the females, but there is no correlation between the reaction severity and the clinical course of the disease.

Eye Manifestations

The eye inflammation, which is one of the most important organ involvement of the syndrome, is encountered in half of the patients. It manifests itself as redness in the eye and blumy vision. As compared to females and elderly, eye disease is more commonly seen in males and younger patients and the course is more severe. The symptoms concerning the eye disease can be various. In the figure on the left, "hypopion" which is the earliest manifestation identified, is shown. Among the patients with the eye disease, about 10-20% end up with loss of vision.

Joint Manifestations

   A year after defining the syndrome, in 1938, Hulusi Behçet added the joint manifestations to the syndrome. Joint involvement, which is seen in almost half of the patients, is one of the major complaint of the patients. It manifest itself as joint pain, but more commonly as joint swelling. Although the pain, swelling and limitation of movement of the joint is common, reddenning is rare. The most common involved joints are the knees, ankles, wrists and elbows in decreasing order. Joint destruction is not seen and it is self-limited to 1-2 weeks.

Vessel Manifestations

   In Behçet’s syndrome, vein involvement is common, whereas that of the arteries is rare. Trombophlebitis is seen in one fourth of the patients, who are mostly males. It is characterised by swelling in the legs. Most commonly it is seen as superficial or deep thrombophlebitis. Especially when the thrombophlebitis is a long-lasting one, slow-healing leg ulcers can be the sequela.

Nervous System Manifestations

   Severe headache, double-vision, numbness and weakness of arms and legs and dizziness can be seen, although rarely.

Gastrointestinal Manifestations

   Stomach pain and diarrhaea can be seen. Lesions in bowels can occur. These findings are rare in Turkey but more common in Japan.

Yalçın Tüzün, M.D., Sebahattin Yurdakul, M.D.
Main Page