Bechet’s disease (BD) is a chronic inflammatory disease of the blood vessels,
BD is a chronic inflammatory disease of the blood vessels, mainly manifested by recurrent oral ulcers, genital ulcers, ophthalmia and skin damage, but also involving the blood vessels, nervous system, digestive 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 has a high incidence in East Asia, the Middle East and the Mediterranean region and is known as Silk Road disease. There is no exact information on the incidence in China, and the disease can occur at any age, with the peak age of onset being 16 to 40 years. In China, the majority of patients are female, and male patients have more vascular, neurological and ocular involvement than females and are more severely affected. Clinical manifestations: 1, oral ulceration Almost all patients have recurrent, painful oral ulceration similar to stomatitis (Aphthous
ulceration, Aphthous ulcer), most patients have this sign as the first symptom. Aphthous ulceration 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 can be the size of a grain of rice or a soybean, round or oval, with clear edges, varying in depth, and a yellow covering at the bottom, surrounded by a red halo with clear edges and pain. About 1~2 weeks can fade on its own without leaving scars. 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 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, penis, etc. 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. Ophthalmitis affects about 50% of patients. Uveitis can appear months or even years after the onset of the disease. Ocular lesions manifest as blurred vision, decreased visual acuity, ocular congestion, ocular pain, photophobia and tearing, foreign body sensation, mosquito flying and headache. It is usually chronic, recurrent and progressive, and both eyes can be involved. The most common ocular lesion is uveitis. All other tissues of the eye can be involved. Keratitis, herpetic conjunctivitis, sclerositis, chorioretinitis, retinitis, optic nerve papillitis, necrotizing retinal vasculitis, and fundus hemorrhage. Anterior chamber pus accumulation is the most severe form of uveitis. Uveitis and retinal vasculitis are the characteristic manifestations of ocular damage. In addition, there may be lens hemorrhage or atrophy, glaucoma, and retinal detachment. Optic disc edema alone suggests cerebral venous thrombosis, intracranial lesions can lead to visual field defects. Skin lesions have a high incidence of skin lesions, up to 80%, with a variety of 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, and pyoderma. A patient may have more than one type of skin lesion. Signs of particular diagnostic value are erythema nodosum-like lesions and inflammatory reactions to minor trauma (needling). 5. Joint damage 25-60% of patients have joint symptoms. It presents as a relatively mild, limited, asymmetric arthritis. It mainly involves the knee and other large joints. The disease sometimes involves the sacroiliac joints in HLA-B27-positive patients and has a similar presentation to that of ankylosing spondylitis.