Recognizing Behçet’s disease

  Behcet’s disease, also known as Behcet’s syndrome and oro-ocular-genital triad. Behcet’s disease, was first reported by Behcet, a Turkish ophthalmologist (1937). Oral ulcers, genital ulcers and uveitis are the three main clinical features of the disease. Chronic, recurrent disease with involvement of multiple systems is characteristic of the disease. Except for a few deaths due to visceral damage, the prognosis of most patients is good, and some patients are left with visual impairment.  (1) Oral ulcers (98.6% with, 55.2% initial symptoms.)  (2) Pubic ulcers (inner part of labia minora and labia majora, vestibular mucosa and around the vaginal opening, cervix.)  (3) Diverse ocular signs (keratitis, anterior chamber pus accumulation, iridocyclitis, chorioretinitis, retinitis, optic neuritis, optic nerve atrophy, conjunctivitis, etc. Ocular damage can often lead to vision loss or even blindness.) In addition, skin lesions, gastrointestinal lesions, vascular lesions, pulmonary lesions, arthritis, urinary lesions, and neurological lesions are also associated.  The main manifestations are: (1) The most common skin signs are erythema nodosum, but also erythema multiforme and acne-like rash, and allergic reactions to skin pricks – small papules or pustules. Such as impetigo, folliculitis, boils, cellulitis and ulcers.  (2) Signs of the cardiovascular system are mainly allergic small-vessel vasculitis, which can manifest occlusive phlebitis, endarteritis, aortitis and aortic valve closure insufficiency, and terminal aneurysms. The lesions can involve veins, ranging from small retinal vessels to large upper and lower vena cava. The occurrence of pulmonary thrombophlebitis can cause pulmonary infarction and recurrent hemoptysis; multiple pulmonary artery thrombosis can cause pulmonary heart disease.  (3) Neurological symptoms account for about 8% to 10%, and the disease is serious and most dangerous. The most common paroxysmal headache is recurrent. Central nervous system thrombophlebitis and microvascular perivasculitis can cause focal softening of brain tissue. Neurological symptoms appear later than other symptoms and may include dizziness, memory loss, severe headache, motor disorders, recurrent paraplegia and total paralysis, and coma. Clinical manifestations include brainstem, meningeal, and encephalitis syndromes and organic psychosis syndromes.  (4) Gastrointestinal lesions occur in about 50% to 64% of cases and can cause ulceration of the entire gastrointestinal tract and mucosa from the mouth to the anus, leading to perforation and proliferative changes, as well as Budd-chiari syndrome due to hepatic vein occlusion, which is often misdiagnosed and increases its harmfulness.  (5) Hyperthermia sepsis-like manifestations although mostly irregular low-grade fever, but some cases appear flaccid hyperthermia with leukocytosis, resembling sepsis.  (6) Joint and muscle symptoms can involve large and small joints of the extremities and the lumbosacral region, with wandering pain, without deformity or bone destruction. The knee, ankle and wrist joints are most frequently involved, with varying degrees of dysfunction, which may return to normal.