Behcet’s disease

The Behcet ‘s disease is a multisystem disease (affecting several systems) of unknown origin, and chronic relapsing course characterized by the presence of vasculitis (inflammation of blood vessels) and clinical symptoms characteristic.

It was described by the Turkish dermatologist Behcet in 1937 and since then it has not yet been possible to find out what the ultimate cause of this disease is.

It is a disease with a worldwide distribution, although it is observed more frequently in the eastern Mediterranean area and Japan. It is somewhat more common in men, in whom it also develops more severely.

The age of onset is most common in the 3rd and 4th decade of life.

Causes of Behcet’s disease

The etiology is unknown, but there are indications of a communicable agent, which has not yet been confirmed.

Immune phenomena, both cellular and humoral in nature, are involved in its pathogenesis, which on the other hand are inconsistent and non-specific. Antibodies against the individual’s own oral mucosa have been detected, for example, in approximately 50% of patients.

On the other hand, the fundamental lesion is a vasculitis (inflammation of the circulatory vessels) with intense cellular infiltration that mainly affects veins and capillaries located around the macroscopic lesions.

What symptoms appear?

The appearance of recurrent canker sores or oral ulcers (present in practically 100% of cases) is characteristic, which are usually the first and most frequent manifestation.

These are herpetiform ulcers, usually very painful, rounded, yellowish and of variable size, which heal in 1 to 3 weeks without leaving a scar.

Another frequent manifestation is genital ulcers (present in 60-80% of cases), which in men are located on the penis, glans and scrotum, and are usually recurrent, large, very painful and heal slowly leaving a scar, and in the In the case of women, they settle on the vulva, vagina and cervix, and can even go unnoticed.

Ocular involvement (60-70%) is generally bilateral, and usually occurs after several years of outbreaks of oral and genital thrush. The most characteristic is anterior uveitis, although the most common is posterior uveitis, which leads to decreased visual acuity and, in 50%, can progress to blindness in about 4 years. Another type of ocular involvement is possible, and thus, choroiditis, vitreous hemorrhages, optic neuritis, etc. are associated to varying degrees.

Skin involvement is frequent (70-80%) and varied, such as erythema nodosum, papulopustular lesions, cutaneous vasculitis, acneiform nodules, folliculitis, sometimes several of them coexisting.

Regarding joint involvement, the presence of arthralgias is more frequent than arthritis , although when these appear they are usually monoarticular or oligoarticular, non-deforming, subacute and self-limited, although recurrent. Knees, ankles, elbows, and wrists are often affected.

Neurological manifestations (4-48%) usually appear between 1 and 7 years after the onset of the disease and are polymorphic, since they can affect any region of the central nervous system (CNS). Diffuse headaches, aseptic meningitis and meningoencephalitis are more frequent , as well as benign intracranial hypertension (HTIC) and hemiparesis and hemiplegia or tetraparesis and tetraplegia. Patients with neurological symptoms can die or be left with sequelae, sometimes disabling.

Gastrointestinal involvement (5-40%) generally consists of episodes of diarrhea, abdominal pain and bloating, nausea, and vomiting. Ulcerative, single or multiple lesions may appear that simulate Crohn’s disease , and cause other complications such as perforation or massive hemorrhage.

Thrombophlebitis and pulmonary embolism (PE) are less common. Other less important manifestations are fever , epididymo-orchitis, and myositis (muscle inflammation).

It is generally a benign disease, but is occasionally associated with serious complications. Many of the patients with posterior segment involvement suffer from severe vision loss. Most fatal cases present neurological, intestinal ulcerative or vascular involvement.

How is it diagnosed?

Laboratory tests are nonspecific and do not help diagnosis, so diagnosis should be based on long-term clinical observation. There are various diagnostic classifications, although this is the most used:

To establish the diagnosis, it is necessary that the first criterion be present along with 2 or more of the rest.

In the case of ocular involvement, the examination should consist of anterior and posterior biomicroscopy and fundus examination.

In neurological manifestations, CSF analysis shows an increase in protein sprain, PMN, lymphocytic or mixed pleiocytosis. Evoked potentials and MRI are the tests with the highest diagnostic yield.

The phenomenon of pathergy or hyperergia (abnormal reaction to an allergic agent) to the injection of subcutaneous physiological saline (25-75% positivities) is also typical.

The differential diagnosis should be made mainly with Reiter’s disease, but also with SLE, recurrent oral aphthosis, Crohn’s disease, ulcerative colitis, multiple sclerosis, etc.

Treatment of Behcet’s disease

The choice of treatment depends on the clinical manifestations and their severity:

• Joint clinic: NSAIDs or Colchicine are used.
• Vascular clinic (thrombophlebitis…): Anticoagulation.
• Cutaneous-mucosal clinic: Colchicine, topical corticosteroids or decoction of plantain are used.
• Eye clinic: Cyclosporine.
• Meningoencifalitis: Corticosteroids.

However, a significant percentage of patients require only minimal care for a good evolution.

How to avoid it?

Currently the true cause of the appearance of Behcet’s disease is not known, so it is not possible to avoid it; It is possible, however, to avoid the appearance of complications with the correct treatment of the clinical manifestations.

Given the appearance of the typical clinical manifestations of this disease, it would be advisable to study, at least control it, by a professional, who will ultimately determine whether it is isolated symptoms or the diagnosis can be made.

On the other hand, in the case of the patient already diagnosed, the appearance of symptoms that indicate complications (alterations in visual acuity, bloody diarrhea, persistent headache with stiff neck and fever, etc.), make it necessary to visit the doctor for new assessment.