Neurology Medicine

In 1937, Dr. Hulusi Behçet, Turkish doctor described a syndrome characterized by a triad of signs: aphthous stomatitis, genital ulcers and uveitis (presence of inflammatory cells in the anterior chamber of the eye).

The recurrent oral thrush (100% of cases) are painful and have a variable size and appearance alone healing in 1-3 weeks without scarring.

The genital sores (60-80%) are located on the glans penis and scrotum in males and the vulva, vagina and cervix in women, being painful and slow to heal in the male, much less troublesome in women. Uveitis, bilateral almost always present in 60-70% of patients. Sometimes associated choroiditis, vitreous hemorrhage, optic neuritis, retinal and other vascular disorders that can lead to blindness if left untreated the disease.

Patients often have more than 20 years and the disease is about twice as common in men than in women. But cases have been reported in newborns of mothers with the disease. Neonatal disease is characterized by aphthous stomatitis and phenomena on the skin that disappear spontaneously after 6 months. The transmission of the disease from mother to child is due to specific antibodies to the fetus through the placenta.

Although Behçet’s syndrome is only rarely diagnosed in children should be considered in the differential diagnosis of any multisystem inflammatory disorder. The clinical picture of Behçet’s syndrome in children differs from adult in the lower frequency of ocular and other manifestations that are less common. These include neutropenia, splenomegaly, Budd-Chiari syndrome, pulmonary infiltrates and ruptured aneurysm of the pulmonary artery.

The systems involved in Behçet’s disease are:

* Oral ulcers: the first symptom observed in 50% to 70% of patients but in the course of the disease are seen in 100% of them.
* Genital sores: they appear in 80% of subjects.
* Cutaneous manifestations: seen in 80% of cases and involve folliculitis, pyoderma, pustules, vesicles, papules, boils, erythema nodosum and like lesions caused by acne. It is observed in the skin pathergy erosion, puncture or intradermal injections of saline (*)
* Implications eye: are manifested in 75% as iridocyclitis or anterior or posterior segment. May be associated with chorioretinitis, optic papillitis and retinal thrombophlebitis). Frequent relapses can cause complications such as glaucoma, cataracts and blindness.
* Joints: the incidence is 65% with arthralgia and arthritis, especially of the knee.
* Venous Implications: typical recurrent thrombophlebitis in Behçet syndrome including superficial thrombophlebitis, intracranial venous thrombosis, occlusion of the inferior or superior vena cava and Budd-Chiari syndrome.
* Implications of CNS have been described only in 18% of cases are expressed as meningitis, pseudotumor cerebri, cranial neuroparálisis pyramidal and extrapyramidal symptoms. Typically the disease begins as multiple sclerosis because of the vasculitis and obstruction of the cranial vessels.
* Implications of the digestive tract: nonspecific symptoms have been observed as cramps, nausea, vomiting, diarrhea and anorexia. Other ulcerative colitis patients show symptoms similar to Crohn’s disease. Esophageal involvement is rare, but when there are to ulcerations that can dig beneath the mucosa, stenosis, fistulas and perforation
* Other systems: it is rarely described growing glomerulonephritis, nephrotic syndrome and amyloidosis. A blood level have been described aortic aneurysms and transient ischemic attacks. At heart, myocarditis, pericarditis and myocardial infarction. Finally also described myositis in Behçet’s syndrome

Source: www.iqb.es/monografia/sindromes/s003_01.htm
image source: www.ispub.com/ispub/ijn/volume_9_number_2_5/behcet_s_disease_presented_with_isolated_peduncular_hallucinosis_a_case_report/behcet-fig1.jpg