Can Stevens-Johnson syndrome present with ocular involvement only, without skin or other mucosal lesions?

Can SJS Present with Isolated Ocular Involvement?

Yes, Stevens-Johnson syndrome can present with isolated ocular involvement without skin lesions, though this is an atypical presentation that occurs primarily in specific clinical contexts.

Clinical Presentation Patterns

While classic SJS involves mucocutaneous disease with skin lesions, ocular inflammation can develop before skin signs appear 1. The UK guidelines explicitly state that "ocular involvement in SJS/TEN usually occurs concurrently with skin disease but may develop before or after the appearance of cutaneous signs" 2.

The "Atypical" or "Incomplete" SJS Phenotype

A distinct clinical entity has been recognized, particularly in Mycoplasma pneumoniae-associated cases, characterized by:

• Predominantly mucous membrane involvement with little or no cutaneous lesions 2
• This variant is termed "Mycoplasma pneumoniae-associated mucositis" 2
• Most commonly affects children 2

However, case reports document this presentation can also occur in:

• Healthy adults after drug exposure (e.g., azithromycin) without mycoplasma infection 3
• Patients presenting with isolated oral mucositis and conjunctivitis without skin or genital involvement 4, 5

Critical Diagnostic Considerations

Early Recognition Challenges

The absence of typical skin findings creates diagnostic pitfalls:

• Ophthalmologists may be the first to encounter these patients, presenting initially with non-specific ocular symptoms like superficial keratitis 6
• The diagnosis requires maintaining clinical suspicion even when the "full house" of features is absent 5
• Consider the entire clinical context: prodromal symptoms (fever, malaise, upper respiratory symptoms), medication history, and temporal progression 6

Key Clinical Features to Identify

When evaluating isolated ocular involvement, look for:

• Purulent keratoconjunctivitis with eyelid edema 2
• Pseudomembrane formation on conjunctival surfaces 2
• Corneal and conjunctival epithelial defects 2
• Chemosis and conjunctival injection 2
• Concurrent oral mucositis (even without skin lesions) 4, 5

Management Implications

Immediate Actions Required

Even with isolated ocular disease:

1. Immediate ophthalmology consultation - daily review is mandatory during acute phase 2
2. Stop any potential culprit drug immediately 2
3. Initiate aggressive ocular supportive care:
   • Two-hourly preservative-free lubricants (hyaluronate or carmellose) 2
   • Daily ocular hygiene by ophthalmologist to prevent conjunctival adhesions 2
   • Topical antibiotics if corneal defenses compromised 2

Long-term Sequelae Risk

Critical caveat: Even with isolated or mild acute ocular involvement, 50-63% of patients develop chronic complications including severe dry eye, symblepharon, and limbal stem cell deficiency 2, 7. The severity of acute ocular disease does not predict long-term complications 2, making prolonged ophthalmologic follow-up essential regardless of initial presentation severity.

Clinical Bottom Line

While uncommon, SJS can present with isolated ocular (and oral mucosal) involvement without skin lesions. This atypical presentation is most associated with Mycoplasma pneumoniae infection in children but has been documented in drug-induced cases in adults. The absence of skin findings should not delay diagnosis or treatment when characteristic mucosal inflammation is present with appropriate clinical context. Early aggressive ophthalmologic management is critical to prevent devastating long-term visual morbidity, which occurs in the majority of patients regardless of acute disease severity.