SJS, Ocular Cicatricial Pemphigoid and Neurotrophic Keratopathy

Last updated: November 2026

Stevens-Johnson syndrome, ocular cicatricial pemphigoid and neurotrophic keratopathy all destroy the ocular surface. Early recognition and aggressive surface protection decide whether the patient keeps useful vision.

Stevens-Johnson syndrome and TEN

Acute mucocutaneous reaction, usually to drugs (sulfonamides, anticonvulsants such as carbamazepine and phenytoin, allopurinol, NSAIDs) or infection.
Acute phase: examine the eyes within 24–48 hours of admission; remove membranes, lubricate, topical steroids and antibiotics; early amniotic membrane transplantation over the lids and ocular surface reduces chronic damage.
Chronic phase: dry eye, trichiasis, lid margin keratinisation, symblepharon, limbal stem cell deficiency. Scleral lenses (PROSE), lid margin mucous membrane grafts, and in selected eyes keratoprosthesis.

Ocular cicatricial pemphigoid (mucous membrane pemphigoid)

Older adults; chronic, bilateral, progressive conjunctival scarring: forniceal shortening, symblepharon, entropion, trichiasis, corneal vascularisation.
Diagnosis: conjunctival biopsy with immunofluorescence (linear IgG, IgA or C3 along the basement membrane).
Systemic immunosuppression is required (dapsone for mild; mycophenolate, cyclophosphamide or rituximab for severe), shared with dermatology. Avoid surgery during active inflammation.
Drug-induced pseudopemphigoid can follow long-term glaucoma drops.

Neurotrophic keratopathy

Mackie stage	Features	Treatment
1	Punctate keratopathy, irregular epithelium, reduced tear break-up	Preservative-free lubricants; stop toxic drops
2	Persistent epithelial defect with smooth rolled edges	Bandage contact lens or autologous serum, punctal plugs, tarsorrhaphy or botulinum-induced ptosis, cenegermin (nerve growth factor) drops
3	Stromal ulcer, melting, perforation risk	Amniotic membrane, glue, tarsorrhaphy; keratoplasty or corneal neurotisation in selected cases

Causes: herpes simplex and zoster, diabetes, trigeminal nerve damage (surgery for acoustic neuroma, stroke), chemical burns, topical anaesthetic abuse, long-term drops. Test corneal sensation in every non-healing epithelial defect.

Where residents slip

Not examining the eyes early in SJS on the medical ward.
Doing fornix surgery in active OCP.
Missing reduced corneal sensation in a non-healing defect.
Continuing toxic preserved drops in neurotrophic disease.

Test yourself: flip cards

Key sources

Shanbhag SS, et al. Multidisciplinary care in Stevens-Johnson syndrome. Ther Adv Chronic Dis. 2020
Mackie IA. Neuroparalytic keratitis. In: Fraunfelder F, et al. Current Ocular Therapy. 1995
Bonini S, et al. Phase II randomized trial of recombinant human nerve growth factor for neurotrophic keratitis. Ophthalmology. 2018;125:1332-1343

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Written by Dr. Dhaval Patel, MD (Ophthalmology, AIIMS New Delhi)

Consultant, Cataract & Refractive Surgery — among a small number of AIIMS-trained ophthalmologists practicing in Gujarat. Read full credentials & experience or view his 28 publications on ResearchGate.

📚 Also the author of OKAT Decoded and other high-yield ophthalmology exam-prep books used by residents and PG aspirants.

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