Post-acute phase and sequelae management of epidermal necrolysis: an international, multidisciplinary DELPHI-based consensus.

BACKGROUND: Long-term sequelae are frequent and often disabling after epidermal necrolysis (Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)). However, consensus on the modalities of management of these sequelae is lacking. OBJECTIVES: We conducted an international multicentric DELPHI exercise to establish a multidisciplinary expert consensus to standardize recommendations regarding management of SJS/TEN sequelae. METHODS: Participants were sent a survey via the online tool "Survey Monkey" consisting of 54 statements organized into 8 topics: general recommendations, professionals involved, skin, oral mucosa and teeth, eyes, genital area, mental health, and allergy workup. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). Results were analyzed according to the RAND/UCLA Appropriateness Method. RESULTS: Fifty-two healthcare professionals participated. After the first round, a consensus was obtained for 100% of 54 initially proposed statements (disagreement index < 1). Among them, 50 statements were agreed upon as 'appropriate'; four statements were considered 'uncertain', and ultimately finally discarded. CONCLUSIONS: Our DELPHI-based expert consensus should help guide physicians in conducting a prolonged multidisciplinary follow-up of sequelae in SJS-TEN.

Management of ocular involvement in the acute phase of Stevens-Johnson syndrome and toxic epidermal necrolysis: french national audit of practices, literature review, and consensus agreement.

Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) can lead to severe ophthalmologic sequelae. The main risk factor is the severity of the initial ocular involvement. There are no recommendations for ocular management during acute phase.We conducted a national audit of current practice in the 11 sites of the French reference center for toxic bullous dermatoses and a review of the literature to establish therapeutic consensus guidelines. We sent a questionnaire on ocular management practices in SJS/ TEN during acute phase to ophthalmologists and dermatologists. The survey focused on ophthalmologist opinion, pseudomembrane removal, topical ocular treatment (i.e. corticosteroids, antibiotics, antiseptics, artificial tear eye drops, vitamin A ointment application), amniotic membrane transplantation, symblepharon ring use, and systemic corticosteroid therapy for ophthalmologic indication. Nine of 11 centers responded. All requested prompt ophthalmologist consultation. The majority performed pseudomembrane removal, used artificial tears, and vitamin A ointment (8/9, 90%). Combined antibiotic-corticosteroid or corticosteroid eye drops were used in 6 centers (67%), antibiotics alone and antiseptics in 3 centers (33%). Symblepharon ring was used in 5 centers (55%) if necessary. Amniotic membrane transplantation was never performed systematically and only according to the clinical course. Systemic corticosteroid therapy was occasionally used (3/9, 33%) and discussed on a case-by-case basis.The literature about ocular management practice in SJS/ TEN during acute phase is relatively poor. The role of specific treatments such as local or systemic corticosteroid therapy is not consensual. The use of preservatives, often present in eye drops and deleterious to the ocular surface, is to be restricted. Early amniotic membrane transplantation seems to be promising.

Panorama of limbal changes.

The corneal limbus is a privileged region on the border between two quite different microenvironments, where corneal epithelial stem cells, numerous melanocytes, and antigen-presenting cells are all concentrated within a richly vascularized and innervated stroma. This situation within the ocular surface confers on it the key functions of barrier, epithelial renewal and defense of the cornea. As an immunological crossroads and since the corneoscleral limbus is directly exposed to external insults such as caustic agents, ultraviolet radiation, microbial agents, and allergens, it is the potential site of many tumoral, degenerative or inflammatory pathologies and may progress under certain conditions to limbal stem cell deficiency.

[Panorama of limbal alterations (French translation of the article)]. / Panorama des altérations limbiques cornéennes.

The corneal limbus is a privileged region on the border between two quite different microenvironments, where corneal epithelial stem cells, numerous melanocytes, and antigen-presenting cells are all concentrated within a richly vascularized and innervated stroma. This situation within the ocular surface confers on it the key functions of barrier, epithelial renewal and defense of the cornea. As an immunological crossroads and since the corneoscleral limbus is directly exposed to external insults such as caustic agents, ultraviolet radiation, microbial agents, and allergens, it is the potential site of many tumoral, degenerative or inflammatory pathologies and may progress under certain conditions to limbal stem cell deficiency.

[Tacrolimus ointment in the management of atopic keratoconjunctivitis]. / Tacrolimus pommade dans la prise en charge de la kératoconjonctivite atopique.

INTRODUCTION: Atopic keratoconjunctivitis is associated with eyelid eczema. It may require the use of local corticosteroids which if prolonged can be a source of ocular complications. Tacrolimus is an immunosuppressant used in cutaneous application in atopic dermatitis. The aim of this study was to measure the efficacy and tolerance of tacrolimus 0.1% ointment in palpebral application in atopic keratoconjunctivitis. PATIENTS AND METHODS: This retrospective, single-center study was conducted between June 2014 and February 2017. Patients with atopic keratoconjunctivitis not controlled by first-line medical treatments were included. The primary endpoint was the evolution of functional signs as assessed by the NEI-VFQ25 and OSDI quality of life scores. Secondary endpoints were visual acuity and local corticosteroid use. RESULTS: Among the 18 patients included, the mean age was 37.9±16.8 years. The first follow-up visit was on average 68.3±55.3 days after initiation of treatment. The NEI-VFQ25 score was significantly improved for seven of its sub-scores (P<0.05) and the mean OSDI decreased significantly from 52.3±26.2 to 22.0±27.0 (P<0.001) showing a decrease in eye discomfort. A significant reduction was observed in the number of patients using local corticosteroids. There was no significant change in visual acuity. CONCLUSION: Tacrolimus ointment 0.1% in palpebral application appears to be an effective treatment for the management of atopic keratoconjunctivitis.

Tacrolimus ointment in the management of atopic keratoconjunctivitis.

INTRODUCTION: Atopic keratoconjunctivitis is frequently associated with atopic eyelid dermatitis. It may require topical steroids, the prolonged use of which may cause ocular complications. Tacrolimus is an immunosuppressant used topically on the skin in atopic dermatitis. The purpose of this study is to evaluate the efficacy and tolerability of tacrolimus 0.1% ointment applied to the eyelids in atopic keratoconjunctivitis. PATIENTS AND METHODS: This is a single center, retrospective study carried out between June 2014 and February 2017. Patients presenting with atopic keratoconjunctivitis uncontrolled by first-line medical treatment were included. The main outcome was change in functional symptoms as evaluated by the NEI-VFQ25 and OSDI quality of life scores. Secondary criteria were visual acuity and topical steroids use. RESULTS: Among the 18 patients included, the mean age was 37.9±16.8years. The first follow-up visit occurred on average 68.3±55.3 days after initiation of treatment. The NEI-VFQ25 score improved significantly for seven of the sub-scores (P<0.05), and the mean OSDI decreased significantly from 52.3±26.2 to 22.0±27.0 (P<0.001), demonstrating a decrease in ocular symptoms. A significant reduction in the number of patients requiring topical steroid treatment was observed. There was no significant change in visual acuity. CONCLUSION: Tacrolimus 0.1% ointment applied to the eyelids appears to be an effective treatment in the management of atopic keratoconjunctivitis.

Scleral lenses for severe chronic GvHD-related keratoconjunctivitis sicca: a retrospective study by the SFGM-TC.

Chronic GvHD-related keratoconjunctivitis sicca (cGvHD-related KCS) can significantly alter the quality of life of patients after allogeneic hematopoietic stem cell transplantation. The aim of this work was to assess the efficacy and tolerability of scleral lenses to treat severe cGvHD-related KCS. In this retrospective, multicenter study, we included 60 consecutive patients diagnosed with cGvHD-related KCS and fitted with scleral lenses. Patients were evaluated at baseline and at 2 months with the following tests: the Ocular Surface Disease Index (OSDI) to assess quality of life, the Oxford score to grade corneal damage and the logarithm of minimal angle of resolution (Log MAR) scale to determine visual acuity. We observed improvement in quality of life in 58 patients (97%). All parameters improved at 2 months. We observed significant differences at 2 months compared with baseline for the mean OSDI (86 versus 30, respectively, P<0.001), the mean Oxford score (3.2 versus 1.3, respectively, P<0.001) as well as visual acuity (Log MAR of 0.33 versus 0.10, respectively, P<0.001). Treatment with scleral lenses was discontinued in only 5 patients (8%) with a median follow-up of 20.5 months (range: 2-125 months). Scleral lenses were very efficient and well tolerated in patients with severe cGvHD-related KCS.

[Gas-permeable scleral lens for management of severe keratoconjunctivitis sicca secondary to chronic graft-versus-host disease]. / Traitement des sécheresses oculaires sévères secondaires à la maladie du greffon contre l'hôte chronique par lentilles sclérales perméables à l'oxygène.

INTRODUCTION: Graft-versus-host disease is a major complication of allogeneic hematopoietic stem cell transplantation. Severe keratoconjunctivitis sicca is common in patients with chronic GVH disease. The goal of this study was to evaluate the safety and efficacy of a gas-permeable scleral lens in the management of severe dry eye disease associated with chronic GVH. PATIENTS AND METHODS: This is a retrospective study from June 2009 to November 2013. Patients fitted with scleral lenses for severe keratoconjunctivitis sicca associated with chronic GVH were included. The main outcomes measured were best-corrected visual acuity and quality of life (OSDI and NEI-VFQ25) composite scores before and six months after scleral lens fitting. RESULTS: Sixteen patients were included. The mean age was 52 years (19-69 years). Mean follow-up was 20 months (3-48 months). All patients reported improvement of their ocular symptoms. Best corrected visual acuity improved from 0.21 ± 0.26 to 0.1 ± 0.14 logMAR (P = 0.002), OSDI score improved from 92.1 ± 11.3 to 23.5 ± 11.2 (P = 0.002) and NEI-VFQ25 improved from 41.3 ± 7 to 83.1 ± 15.9 (P = 0.003), 6 months after scleral lens fitting. No serious adverse events, infectious, hypoxemic or allergic complications attributable to the scleral lens occurred. CONCLUSION: Gas-permeable scleral lens use appears to be safe and effective in patients with severe dry eye related to chronic GVH.

Efficacy and safety of pre-Descemet's membrane sutures for the management of acute corneal hydrops in keratoconus.

AIMS: The aim of this study was to assess the efficacy and safety of pre-Descemet's membrane (DM) sutures associated with intracameral air injection for management of acute corneal hydrops associated with keratoconus. METHODS: We carried out a retrospective interventional study of seven consecutive cases. Three to seven pre-DM sutures with 10-0 nylon were applied perpendicularly to the tear as close as possible to DM, under general anaesthesia. An air bubble was injected into the anterior chamber at the end of each procedure. RESULTS: Corneal oedema began to decrease from day 1 after surgery in all our patients. Best-corrected visual acuity progressed from 2.13 to 1.65 logMar (p=0.031) 1 month after surgery, and from 2.13 to 0.84 logMar (p=0.016) 2 months after surgery. The mean corneal thickness measured by anterior segment optical coherence tomography decreased from 1472 µm (range 689-2770 µm) on day 0 to 909 µm (range 484-1640 µm) on day 1 (p=0.016), 716 µm (range 484-1380 µm) on day 15 (p=0.016) and 528 µm (range 404-618 µm) 1 month after surgery. CONCLUSIONS: Our results suggest that intrastromal pre-DM sutures and intracameral air injection could promptly restore imperviousness of posterior stroma. This technique seems to be a safe and useful procedure to shorten acute corneal hydrops.

[Bringing back scleral contact lenses]. / Vers une réhabilitation des verres scléraux?

PURPOSE: Although old generations of scleral contact lenses were totally replaced by soft and hard contact lenses, new designs and materials have recently allowed the successful use of nonfenestrated scleral contact lenses in patients who are intolerant to regular contact lenses. These new scleral contact lenses form a bridge over the cornea, which is thus permanently bathed in a saline reservoir. METHODS: Retrospective study in patients suffering from corneal diseases altering the vision and/or responsible for ocular discomfort, and who are intolerant to conventional contact lenses. RESULTS: Fifteen eyes (10 patients) were fitted with specially designed scleral contact lenses. The mean follow-up was 16 months (range, 1-31 months). The scleral lenses were indicated for optical and/or therapeutic reasons: keratoconus (3 eyes), corneal grafts (7 eyes), refractive surgery (2 eyes), corneal dysplasia (2 eyes), and Stevens-Johnson syndrome (1 eye). Optical results and tolerance were excellent except for one eye. CONCLUSION: New nonfenestrated scleral contact lenses provide a good alternative for patients who are intolerant to conventional contact lenses.