Did you know that erythema multiforme can crawl all over the skin and mucous membrane?
At the beginning, the skin was only a mild blister lesion, which further developed into toxic epidermal necrosis and dissolution.
Did you know that this disease will be more obvious in the eyes?
The lesion will involve the cornea, palpebral conjunctiva, bulbar conjunctiva and eyelids, causing corneal ulcer and anterior uveitis, moderate to severe keratitis or total ophthalmia until blindness
This is the legendary Stevens Johnson syndrome, which is the most serious adverse drug reaction with skin damage and threatening the lives of patients.
In Asian countries, the incidence of SJS is about 8/1million per year. Although the incidence rate is low, there are two terrible things about this disease:
① Every age group has the possibility of getting sick
② More than 50% of the patients were complicated with ocular surface damage. The late treatment effect was poor and the prognosis was poor.
At this point, you may be confused. It doesn't matter. Let's take a few groups of pictures today and simply [analyze] this terrible autoimmune disease according to the eye surface damage classification of SJS patients.
Generally, eye surface damage of SJS patients is divided into three levels: mild eye surface damage, moderate eye surface damage and severe eye surface damage. What are the clinical symptoms of these three levels of patients and how to treat them? You look down.
SJS with mild ocular surface damage
A. The cornea is transparent without epithelial punctate opacity
B. Corneal edema, skin opacity
C. The corneal fluorescence staining showed punctate staining
D. Palpebral conjunctiva is congested and edematous, and pseudomembrane can be seen
When the ocular surface damage of SJS patients is mild, there are few signs and mild symptoms. However, the current ocular surface inflammation cannot be ignored.
Studies have confirmed that infection and autoimmunity can not only cause ocular surface inflammation, but also induce corneal and conjunctival epithelium to produce a variety of inflammatory chemokines, further expanding the ocular surface immune response.
If not controlled, the inflammatory reaction will cause different degrees of ocular surface damage, including squamous metaplasia, limbal stem cell defect and even lacrimal gland function decompensation, which in turn aggravates ocular surface inflammation and forms a vicious circle.
Treatment reference: the patients with mild SJS complicated with eye damage need to supplement tears in the acute stage. Flumiolone is anti-inflammatory. After the inflammation is controlled, the drug can be stopped. Routine eye follow-up.
SJS with moderate ocular surface damage
A. Abnormal eyelid position, entropion with trichiasis
C. Corneal limbal neovascularization grows in, but does not invade the pupil area
D. Conjunctival hyperemia, visible corded conjunctival scar, and the range of keratosis at the facial margin is greater than 1/3 and less than 1/2 of the entire palpebral margin
The treatment of moderate ocular surface damage is mainly to alleviate dry eye symptoms, reduce ocular surface inflammation, protect and repair corneal epithelium.
Dry eye is the most common complication in the chronic phase of SJS. Dry eye caused by long-term chronic inflammation in SJS patients is often accompanied by corneal epithelial damage. At this time, artificial tears without preservatives can be used to prevent the toxic components in preservatives from aggravating the damage of diagonal conjunctival epithelium.
It is observed that the conjunctival scar of patients with moderate ocular surface damage is obvious, and the mechanical damage of trichiasis and palpebral margin to the cornea is inevitable. Therefore, it is necessary to remove trichiasis and wear corneal contact lens when necessary, so as to provide a relatively stable ocular surface environment for epithelial repair.
Treatment reference: SJS complicated with moderate ocular surface damage needs to be supplemented with tears. Fluoromilone is anti-inflammatory and cyclosporine inhibits immune response. In case of severe inflammation, corneal contact lens is used to protect corneal epithelium, and autologous serum promotes repair.
SJS complicated with severe ocular surface damage
A. Face ball adhesion, eyelid position change, limited eye movement
B. White corneal scar in pupil area, with plenty of neovascularization
C. Corneal perforation
D. Continuous patches of conjunctival scar, more than 1/2 of the palpebral margin keratosis.
SJS complicated with severe eye damage, in addition to the structural and functional abnormalities of the ocular surface such as cicatricial ectropion or entropion, trichiasis, and even different degrees of corneal conjunctivalization, persistent or repeated epithelial defects, and repeated attacks of corneal ulcer, eventually developed into corneal perforation.
Once the ocular surface is damaged to this extent, surgery is needed to restore the anatomical structure and physiological characteristics of the ocular surface, so as to rebuild the cornea and conjunctival epithelium.
When SJS is complicated with severe eye damage, the patient's ocular surface condition is very poor, and conventional corneal transplantation is unable to recover. Artificial cornea implantation is the only choice for such patients. So far, only the Michel artificial cornea can be treated.
Microk is made of artificial materials without donor cornea. It will not lead to operation failure due to immune rejection after operation. It is suitable for patients with bilateral corneal blindness who are difficult to succeed in corneal transplantation, including corneal transplantation failure, severe keratoconjunctival scar vascularization, eyelid atresia, and serious autoimmune diseases (such as Stevens Johnson syndrome and cicatricial pemphigoid) caused by chemical injury, thermal burn, explosion injury, etc, Corneal blindness caused by end-stage dry eye.
Due to the low clinical incidence rate of SJS, it is not easy to diagnose SJS in the early stage. In addition, its initial symptoms are diverse, and the initial symptoms such as general discomfort, slight fever, sore throat, etc. are no different from the common cold. Therefore, it will mislead doctors and increase the difficulty of early diagnosis. So once the diagnosis is made, we should attach great importance to it and immediately stop all suspicious drugs and drugs with similar structures.
① Clinical study of Stevens Johnson syndrome with ocular surface damage
② Clinical analysis of 18 cases of Stevens Johnson syndrome Lei Yunhong, sunchaowen, Jian wanai, Wangyi, Tang Fei
③ Research Progress on pathogenesis of Stevens Johnson syndrome he Xuelian (review) liuzhisheng (reviser)