Behcet’s disease (BD) is a systemic, chronic, vascular inflammatory disease that mainly presents with recurrent oral ulcers, genital ulcers, ophthalmia and skin damage, but can also involve the vasculature, nervous system, gastrointestinal tract, joints, lungs, kidneys, epididymis and other organs. Most patients have a good prognosis, but those with ocular, central nervous system and large blood vessel involvement have a poor prognosis. The disease is most common in the age group of 16 to 40 years. It is more common in women than in men.
1. Oral ulcers
Almost all patients have recurrent, painful oral ulceration (Aphthous ulceration), and most patients have this as the first symptom. The ulcers can occur anywhere in the mouth, mostly on the edge of the tongue, cheeks, lips, soft palate, pharynx, and tonsils. They can occur singly or in batches, and are the size of a grain of rice or a soybean, round or oval, with clear edges and varying depths, with a yellow covering at the bottom and a red halo with clear edges around it, which fades on its own without leaving a scar after about 1 to 2 weeks. In severe cases, the ulcers are deep and slow to heal and may occasionally leave scars. Recurrent oral ulcers are the most basic necessary symptom for the diagnosis of this disease.
2. Genital ulcers
About 75% of patients have genital ulcers, and the lesions are basically similar to oral ulcers. However, the number of occurrences is less. The ulcers are deep and large, with severe pain and slow healing. The affected areas are vulva, vagina, perineum, cervix, scrotum and penis. Vaginal ulcers may be painless with only increased discharge. Some patients may bleed due to deep ulcers or rupture of the scrotal vein wall due to necrosis.
3. Ophthalmia
About 50% of patients are affected, and both eyes can be involved. Ocular lesions can appear months or even years after the onset of the disease, which manifests as blurred vision, decreased visual acuity, ocular congestion, ocular pain, photophobia and tearing, foreign body sensation, mosquito flying and headache. The disease usually has a chronic, recurrent, progressive course. Ocular involvement can cause blindness in up to 25% of cases and is the leading cause of disability in this disease. The most common and severe ocular lesion is uveitis. Anterior uveitis, or iridocyclitis, may or may not be associated with pus accumulation in the anterior chamber, while posterior uveitis and uveitis are the main causes of visual impairment. All remaining tissues of the eye can be involved, with keratitis, herpetic conjunctivitis, scleritis, chorioretinitis, retinitis, optic nerve papillitis, necrotizing retinal vasculitis, and fundus hemorrhage. In addition, there may be lens hemorrhage or atrophy, glaucoma, and retinal detachment. Optic disc edema alone suggests cerebral venous thrombosis, and intracranial vasculopathy caused by leukoaraiosis can lead to visual field defects.
4.Dermal lesions
The incidence of skin lesions is high, up to 80%~98%, with various manifestations, including erythema nodosum, herpes, papules, acne-like rash, erythema multiforme, erythema annulare, necrotizing tuberculosis rash-like damage, herpetic necrotizing vasculitis, Sweet’s disease-like lesions, pyoderma, etc.
5.Joint damage
25-60% of patients have joint symptoms. The manifestation is relatively mild, limited, asymmetric arthritis. HLA-B27-positive patients may have sacroiliac joint involvement, which is similar to ankylosing spondylitis.
6.Nervous system damage
Also known as neuroleukopenia, the incidence is about 5% to 50%. It often appears several months to years after the disease, and a few (5%) may be the first symptoms.
7.Digestive tract damage
Also known as intestinal leukoencephalopathy. The incidence is 10% to 50%. The ulcers can be single or multiple, with varying depths, and can be found in the lower esophagus, stomach, distal ileum, ileocecal region, ascending colon, but the ileocecal region is more common. Clinical manifestations may include epigastric fullness, belching, dysphagia, middle and lower abdominal fullness, vague pain, paroxysmal colic, diarrhea, black stool, and constipation. In severe cases, there may be ulcer perforation and even death due to complications such as hemorrhage.
8.Vascular damage
The basic lesion of this disease is vasculitis, which can involve all large and small blood vessels in the body, and about 10%-20% of patients are combined with large and medium-sized vasculitis, which is the main cause of death and disability.
9.Pulmonary damage
The incidence of lung damage is low, about 5%-10%, but most of the disease is serious. Pulmonary aneurysm can be formed when the pulmonary vessels are involved, and when the aneurysm ruptures, pulmonary vascular-bronchial fistula can be formed, resulting in intrapulmonary hemorrhage; pulmonary venous thrombosis can cause pulmonary infarction; alveolar pericapillaritis can cause endothelial proliferation and fibrosis, affecting the function of gas exchange. Patients have cough, hemoptysis, chest pain, and dyspnea when the lungs are involved. Massive hemoptysis can lead to death.
There are no specific serological and pathological features of the disease, and the diagnosis is mainly based on clinical symptoms. For recurrent oral ulcers (3 recurrent episodes in 1 year), the possibility of this disease should be alerted.
There is no recognized effective cure for this disease. A variety of drugs are effective, but most are prone to recurrence after discontinuation. The aim of treatment is to control the existing symptoms, prevent and control important organ damage, and slow down disease progression.
1.General treatment
During the acute activity period, bed rest should be given. During the interictal period, attention should be paid to the prevention of recurrence. For example, control the infection in the mouth and pharynx and avoid irritating food. The corresponding treatment is feasible for those with infection.
2.Local treatment
Oral ulcers can be treated locally with glucocorticoid cream, borax, tin, etc. Genital ulcers can be cleaned with 1:5000 potassium permanganate followed by antibiotic ointment; ocular conjunctivitis and keratitis can be treated with corticosteroid ophthalmic ointment or eye drops; ocular uveitis must be treated with pupil dilators to prevent post-inflammatory adhesions; severe ophthalmitis can be treated with subconjunctival injection of adrenocorticosteroid.
3.Systemic treatment
Mainly non-steroidal anti-inflammatory drugs, hormones, immunosuppressants, biological agents and other drug therapy.