New Grading System and Treatment Guidelines for the Acute Ocular Manifestations of Stevens-Johnson Syndrome

doi:10.1016/j.ophtha.2016.04.041

Ophthalmology

Volume 123, Issue 8, August 2016, Pages 1653-1658

Presented at: World Cornea Congress VI, 2010, Boston, Massachusetts.

https://doi.org/10.1016/j.ophtha.2016.04.041 Get rights and content

Purpose

To describe a new grading system and associated treatment guidelines for the acute ocular manifestations of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN).

Design

Prospective case series.

Participants

Seventy-nine consecutive patients (158 eyes) evaluated and treated for acute ocular involvement in SJS or TEN during hospitalization.

Methods

Photographic and chart review of acute ocular findings, interventions received, and outcomes with regard to visual acuity, dry eye symptoms, and scarring sequelae at least 3 months after the acute illness.

Main Outcome Measures

Visual acuity, dry eye severity, and scarring of the ocular surface and eyelids were assessed after follow-up of at least 3 months.

Results

Cases graded as mild or moderate were managed medically. All had best-corrected visual acuity (BCVA) of 20/20, no dry eye symptoms, and no scarring sequelae. Cases graded as severe or extremely severe were treated with urgent amniotic membrane transplantation (AMT) in addition to medical management. Severe cases all had BCVA of 20/20 and mild or no dry eye problems. Five of 28 patients had mild tarsal conjunctival scarring. No other scarring sequelae occurred. Nine of the 10 extremely severe cases had BCVA of 20/20 (1 was 20/30). Three of 10 had moderate scarring of the tarsal conjunctiva and lid margins and also moderate dry eyes with severe photophobia. Seven of 10 had only mild or no dry eye symptoms and scarring sequelae.

Conclusions

This grading system facilitates decision making in the evaluation and management of the acute ocular manifestations of SJS and TEN. Mild and moderate cases have a low risk of significant scarring or visual sequelae and may be monitored and treated medically if not worsening. Severe and extremely severe cases should receive urgent AMT to decrease the risk of scarring and visual sequelae.

Section snippets

Study Population

Photographs and clinical records of 158 eyes of 79 consecutive patients diagnosed with SJS or TEN were reviewed after approval by the Colorado Multiple Institutional Review Board. All patients were treated at the University of Colorado Hospital or the Children's Hospital of Colorado between June 2006 and July 2013. The extent and location of epithelial sloughing, as determined by fluorescein staining, was documented in each case. The treatments received were noted, and the visual outcomes and

Results

Results are summarized in Table 2.

Discussion

Because of the rarity of SJS and TEN, most ophthalmologists do not treat acute-phase patients on a regular basis. Given the potentially devastating visual problems, however, it is imperative that all ophthalmologists be aware of the current methods for the evaluation and treatment of the disease. The previous lack of clear criteria for the assessment of the acute eye findings has made clinical decision making difficult and has prevented a cohesive method for evaluating and treating these

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Cited by (63)

Clinical Outcome of Amniotic Membrane Transplant in Ocular Stevens–Johnson Syndrome/Toxic Epidermal Necrolysis at a Major Burn Unit
2023, American Journal of Ophthalmology
To analyze the clinical outcome of amniotic membrane transplantation in patients with ocular Stevens–Johnson syndrome/toxic epidermal necrolysis at a major burn unit.
Retrospective, non-randomized interventional study.
A retrospective chart review from April 2014 to January 2022 of 43 patients (85 eyes) at a burn center who underwent amniotic membrane transplantation (AMT) for severe ocular Stevens–Johnson Syndrome (SJS), toxic epidermal necrolysis (TEN), or SJS/TEN was performed. Data regarding the clinical course and outcome were obtained. A comparison between the use of cryopreserved AMT rings (cryoAMT) and dehydrated AMT (deAMT) was also assessed.
A total of 85 eyes in 43 patients underwent AMT for severe ocular SJS/TEN. Of the eyes, 72 received deAMT with symblepharon ring, whereas 13 received cryoAMT over the cornea surface. All patients had deAMT placed over the eyelid margins and palpebral conjunctivae and tucked into the fornices. The average best-corrected visual acuity (BCVA) on last follow-up examination was 20/33, 20/30, and 20/34 in all eyes, the cryoAMT group, and the deAMT group, respectively (no significant difference between groups). The most common suspected inciting agent was lamotrigine (17% of all cases). The average long-term complication score was 1.4, with no significant difference between the cryoAMT group (1.6) and the deAMT group (1.4, P = .5). Symblepharon formation was seen more in the cryoAMT group compared to the deAMT group (P < .05).
The use of AMTs in severe ocular SJS/TEN greatly mitigates long-term complications and improves visual outcome. The retrospective nature of this study limits substantial conclusions regarding any significant difference in outcome between AMT treatment methods. Nevertheless, the use of cryopreserved AMT rings has a similar outcome profile compared to use of dehydrated AMTs with symblepharon ring. Further research is needed to evaluate optimal AMT techniques.
Ocular involvement in Mycoplasma induced rash and mucositis: A systematic review of the literature
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A comprehensive treatment protocol for ocular involvement in SJS/TEN, a related but distinct disease as described above, has previously been described by our group [64] and has been utilized in the treatment of ocular disease in MIRM as well [31]. Gise et al. modified our protocol by suggesting that, in patients with moderate disease, ophthalmologists employ the use of a combined antibiotic-steroid eye drop (tobramycin 0.3%-dexamethasone 0.1%) four times a day instead of a topical fluoroquinolone and steroid separately to reduce drop burden in pediatric patients [30,64,65]. For patients with no ocular involvement, they recommended prophylactic artificial tears.
Mycoplasma pneumoniae induced rash and mucositis (MIRM) is a relatively newly identified clinical entity which is characterized by mucocutaneous manifestations in the setting of Mycoplasma infection. Though a clinically distinct disease, MIRM exists on a diagnostic continuum with entities including erythema multiforme, Stevens-Johnson syndrome, toxic epidermal necrolysis, and the recently described reactive infectious mucocutaneous eruption (RIME). In this systematic review, we discuss published findings on the epidemiology, clinical manifestations, diagnosis, and management of MIRM, with an emphasis on ocular disease. Lastly, we discuss some of the most recent developments and challenges in characterizing MIRM with respect to the related diagnosis of RIME.
Design of functional decellularized matrix for conjunctival epithelial stem cell maintenance and ocular surface reconstruction
2022, Materials and Design
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These results indicated that the stem cell niche provided by DM-SCF realized the important function of maintaining the characteristics of the CjESCs by regulating the activation levels of Wnt/β-catenin and Notch pathways.Fig. 7.. Common and refractory ocular surface diseases, including Steven–Johnson syndrome, acid or alkali chemical injury, and heat burn, can cause severe symblepharon, and large conjunctival defects may occur after the surgical separation of the conjunctiva [37,38]. Conjunctival reconstruction is required to avoid re-adhesion.
Conjunctival defects severely impair ocular surface homeostasis and vision. However, the existing conjunctival reconstruction methods are still unable to achieve satisfactory functional replacement of conjunctiva. Scaffold materials that provide a more stem-cell-friendly microenvironment are in urgent need of improvement. Herein we reported a functional conjunctival reconstruction scaffold constructed from decellularized matrix derived from rabbit subconjunctival fibroblasts (DM-SCF) loaded with conjunctival epithelial stem cells (CjESCs) for ocular surface repair. The DM-SCF had a thickness and mechanical strength close to that of normal conjunctiva and showed excellent biocompatibility. CjESCs inoculated on DM-SCF maintained good stem cell properties, which showed long-term maintenance of low differentiation and good proliferation activity. DM-SCF transplantation with CjESCs showed satisfactory results in rabbit models of a large conjunctival defect. The conjunctiva was reconstructed with abundant goblet cell expression and regular fiber arrangement. Protein mass spectrometry revealed the active extracellular matrix components of DM-SCF, regulating the activation of Wnt/β-catenin and Notch pathways for stem cell maintenance. Overall, our study developed a promising new strategy for ocular surface reconstruction by optimizing bioactive scaffold and providing functional stem cell niche, so as to achieve long-standing and superior functional repair.
Ophthalmic findings of Mycoplasma-induced rash and mucositis (MIRM) distinct from Stevens-Johnson syndrome
2021, Journal of AAPOS
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Ocular treatment often includes aggressive lubrication and combination topical steroid-antibiotic drops. The use of amniotic membrane grafting has become an essential treatment modality in an effort to prevent irreversible scarring even in the early stages of the disease.9 EM shows similarities to MIRM in both patient presentation and epidemiology.
To identify ophthalmic manifestations of Mycoplasma-induced rash and mucositis (MIRM).
The medical records of patients with MIRM treated by the inpatient ophthalmology consult service at a single institution over a period of 4 years were reviewed retrospectively. Eye and skin findings, treatment, hospital course, and follow-up findings were documented and reviewed.
MIRM was identified in 10 patients, 10-20 years of age (mean, 13.) All presented with oral mucosal findings and a prodrome. On initial examination, conjunctival epithelial defects were present in 2 patients; pseudomembranes, in 3. Five patients experienced progressive ophthalmic manifestations during hospitalization, including a new cornea epithelial defect in 1 patient and a new conjunctival epithelial defect in 3 patients. All were treated with topical steroids and antibiotics. Post-discharge follow-up obtained for 7 patients documented good visual outcomes and lack of significant ocular sequelae without requiring invasive intervention.
MIRM, previously referred to as mycoplasma-associated Stevens-Johnson syndrome (SJS) among other names, is now understood to be distinct from SJS in the dermatology community. Our MIRM patients present with mild eye findings and required no surgery, similar to the mild course published in the dermatology literature. Patients with MIRM may have a more benign course than those with SJS.
Vulvovaginal and ocular involvement and treatment in female patients with Stevens–Johnson syndrome and toxic epidermal necrolysis: A review
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Persistence of epithelial defects poses a risk for infection and scarring (Gregory, 2016). Treatment is advanced based on disease severity, as outlined in Table 3 (Gregory, 2016; Power et al., 1995; Thorel et al., 2020). Medical treatment of acute ocular involvement in SJS /TEN is directed toward aggressively reducing inflammation with systemic immunosuppression and/or topical steroid therapy depending on the severity of the disease.
Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious adverse cutaneous drug reactions, characterized by epidermal detachment and mucous membrane involvement. SJS/TEN is more common in female patients, with unique findings in the ocular and vulvar regions. Early recognition and intervention, as well as long-term follow-up, are crucial to prevent devastating scarring and sequelae. This review examines the vulvar and ocular manifestations of SJS/TEN and describes the current treatment recommendations for female patients, requiring close consultation and collaboration among dermatology, ophthalmology, and gynecology.
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: Financial Disclosure(s):

: The author(s) have no proprietary or commercial interest in any materials discussed in this article.

: Author Contributions:

: Conception and design: Gregory

: Analysis and interpretation: Gregory

: Data collection: Gregory

: Obtained funding: none

: Overall responsibility: Gregory

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Original articleNew Grading System and Treatment Guidelines for the Acute Ocular Manifestations of Stevens-Johnson Syndrome

Purpose

Design

Participants

Methods

Main Outcome Measures

Results

Conclusions

Section snippets

Study Population

Results

Discussion

Ophthalmology

Ophthalmology

J AAPOS

Surv Ophthalmol

Ophthalmology

Can J Ophthalmol

J AAPOS

Ophthalmology

Ophthalmology

Original article
New Grading System and Treatment Guidelines for the Acute Ocular Manifestations of Stevens-Johnson Syndrome