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December 05, 2025
4 min read
Stevens-Johnson syndrome is a mucocutaneous immune-mediated hypersensitivity reaction. It is often triggered by drugs or infections. It presents as a skin and mucous membrane drug hypersensitivity reaction with variable severity.
Stevens-Johnson syndrome (SJS) is a mild variant that affects less than 10% of the body surface area whereas toxin epidermal necrolysis is a potentially life-threatening severe form that involves more than 30% of the body surface area. The estimated incidence of SJS is one to six cases per million person-years.
Source: Dhivya Ashok Kumar, MD, FRCS, FICO, FAICO, Nikhil Yadav, DO, DNB , and Amar Agarwal, MS, FRCS, FRCOphth
Precipitating factors
The main etiology is drug induced in about 75% of cases, with 25% of cases attributed to other causes. Drugs are mainly antibiotics, anticonvulsants, NSAIDs, barbiturates, allopurinol and some antidepressants. Other etiologies include viral infections such as HIV, herpes simplex virus, influenza, Epstein-Barr virus, coxsackievirus and COVID-19. Bacterial causes include mycoplasma pneumoniae, typhoid, diphtheria and group A streptococcus. Other causes are fungal, protozoal and idiopathic. Because most patients show symptoms 3 weeks after infection, the etiology is often unknown. HLA-related allele has also been linked to drug-related trigger.
Pathogenesis
SJS is a type IV T-cell-mediated delayed hypersensitivity reaction. Natural killer cells and cytotoxic T cells cause keratinocyte death. Apoptosis of keratinocytes causes epidermis separation from the dermis, leading to skin and mucous membrane separation and development of blisters and erosions. It begins with a prodromal phase with flu-like symptoms such as fever, cough and malaise. The acute phase starts 3 to 5 days after the initial drug administration. Systemic features include classic “target” skin lesions, hemorrhagic inflammation of the mucous membrane (lip crusting), bullae, erosion, and ulceration of the skin, oral, genital and ocular mucosa.
Source: Dhivya Ashok Kumar, MD, FRCS, FICO, FAICO, Nikhil Yadav, DO, DNB, and Amar Agarwal, MS, FRCS, FRCOphth
Ocular involvement
In the acute phase of ocular SJS, clinical pictures include variable papillary conjunctivitis (Figure 1), hemorrhagic blisters on the lids, conjunctival membranes and pseudomembranes, crusting of the lids and skin lesions. Corneal involvement includes dry keratopathy, infectious keratitis and, rarely, perforation.
In sequelae, it can present as a persistent epithelial defect, keratitis, corneal haze and limbal stem cell deficiency. On the ocular surface, symblepharon formation (Figures 2a and 2b), ankyloblepharon, punctal occlusion, medial canthal webbing, lid adhesions and limitation of extraocular movement can occur. Chronic dry eye leading to recurrent ocular inflammation, pain and epiphora can be noted. Dry eye happens due to loss of goblet cells from conjunctival metaplasia and also due to fibrosis of lacrimal gland ductules. Chronic limbal stem cell deficiency can lead to conjunctivalized cornea and corneal opacity with vascularization (Figure 3). Lid changes include cicatricial entropion, ectropion, punctal stenosis, keratinized lid margin, trichiatic lashes (Figure 4), metaplastic lashes and ptosis.
Management
Management of ocular SJS begins with identification and cessation of the causative agent. Most patients are acutely treated in tertiary health care centers for systemic complaints, with referral for acute or chronic sequelae of SJS. Supportive care with fluids, electrolytes and nutritional management along with dermatological management usually happens before referral to the ophthalmologist. Systemic steroids and immunomodulators are given in some patients at referral.
In the acute ocular phase, copious lubricants are advised. Carboxymethylcellulose 1% and highly viscous gel formulations are applied frequently. Topical antibiotics such as moxifloxacin eye drops or gels are preferred for prophylaxis. In acute conditions of conjunctivitis with papillary reaction, milder topical steroids such as fluorometholone are preferred. Lubrication with a damp sterile cotton bud in the fornices is performed regularly on an outpatient basis to prevent symblepharon formation. Rarely, a symblepharon ring or scleral contact lenses are placed to prevent adhesion. Secondary bacterial keratitis is often treated with microbiological confirmation and topical antibiotics.
In eyes with persistent symblepharon, extraocular movement restriction and subsequent diplopia can occur. Symblepharon excision with amniotic membrane grafting is useful in such cases. Punctal dilation is performed in eyes with minimal stenosis. Rarely, punctoplasty is needed. In eyes with mild trichiatic lashes, epilation is performed on an outpatient basis. However, in recurrent trichiasis and metaplastic lashes, electroepilation is preferred. In eyes with entropion with a shortened fornix, mucous membrane grafting is useful. The graft is obtained from oral buccal mucosa. Regular follow-up and frequent lubrication should be advised for all patients. Systemic immunomodulators may be needed in some patients, and long-term prognosis depends on the severity of ocular involvement. In eyes with severe dry eye with patent puncta, punctal plugs can placed. Limbal stem cell transplantation can be performed in eyes with a guarded prognosis with poor vision and corneal haze.
Conclusion
SJS is a life-threatening disorder with multisystem involvement. Early identification and intervention can reduce the morbidity rate and secondary complications occurring from the disease. A multidisciplinary approach with the ophthalmologist, dermatologist and physician will go hand in hand to treat the mildest to most severe form of the disease.
- For more information:
- Amar Agarwal, MS, FRCS, FRCOphth, director of Dr. Agarwal’s Eye Hospital and Eye Research Centre, is the author of several books, including Phaco Nightmares: Conquering Cataract Catastrophes, Bimanual Phaco: Mastering the Phakonit/MICS Technique, Dry Eye: A Practical Guide to Ocular Surface Disorders and Stem Cell Surgery and Presbyopia: A Surgical Textbook. He can be reached at aehl19c@gmail.com; website: www.dragarwal.com.
Sources/Disclosures
Source:
Expert Submission
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Disclosures:
No products or companies that would require financial disclosure are mentioned in this article.
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