OBJECTIVE: To study the outcomes of minor salivary gland transplantation for severe dry eye disease secondary to chronic Steven Johnson Syndrome.
METHODS: It was an ambispective, interventional case series conducted at Rajendra Prasad Centre for Ophthalmic Sciences, Delhi, India from 2022 to 2023 evaluating the outcomes of minor salivary gland transplantation with anchorage of the minor salivary glands to superior rectus muscle in twenty cases of severe dry eye disease secondary to chronic Steven-Johnson Syndrome. The pre-operative clinical parameters were compared to those at post-operative 1 year follow-up.
RESULTS: At 1 year follow-up, there was an improvement in mean Schirmer-1 value (p = 0.0004), hyperemia score (p = 0.0004), keratinization score (p = 0.04), corneal epithelial defect score (p = 0.0004), corneal opacification score (p = 0.001), corneal neovascularization score (p = 0.001), palisades of Vogt score (p = 0.007), corneal keratinization score (p = 0.04) and corneal conjunctivalization score (p = 0.08).
CONCLUSIONS: The minor salivary gland transplantation is a viable management option for cases with severe dry eye disease secondary to chronic Steven Johnson Syndrome with clinical improvement in corneal and conjunctival parameters of the ocular surface.

Toxic Epidermal Necrolysis (TEN) is a rare, but potentially fatal mucocutaneous reaction, that may occur due to an immunologic response to certain medications. However, TEN triggered by Trastuzumab is extremely rare. Early diagnosis, recognition, and prompt cessation of the offending drugs and initiation of steroid therapy with supportive management are the most important actions for managing TEN. Although rare, it is important to be vigilant about this potential adverse reactions associated with trastuzumab to ensure patient safety and contribute to better outcomes.

BACKGROUND: Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious conditions that carry a high rate of morbidity and mortality.
OBJECTIVE: This review highlights the pearls and pitfalls of SJS/TEN, including presentation, diagnosis, and management in the emergency department (ED) based on current evidence.
CONCLUSIONS: SJS/TEN is a rare, delayed hypersensitivity reaction resulting in de-epithelialization of the skin and mucous membranes. The majority of cases are associated with medication or infection. Clinicians should consider SJS/TEN in any patient presenting with a blistering mucocutaneous eruption. Evaluation of the skin, mucosal, pulmonary, renal, genital, and ocular systems are essential in the diagnosis of SJS/TEN, as well as in the identification of complications (e.g., sepsis). Laboratory and radiological testing cannot confirm the diagnosis in the ED setting, but they may assist in the identification of complications. ED management includes stabilization of airway and breathing, fluid resuscitation, and treatment of any superimposed infections with broad-spectrum antibiotic therapy. All patients with suspected SJS/TEN should be transferred and admitted to a center with burn surgery, critical care, dermatology, and broad specialist availability.
CONCLUSIONS: An understanding of SJS/TEN can assist emergency clinicians in diagnosing and managing this potentially deadly disease.

Toxic epidermal necrolysis (TEN) is a life-threatening mucocutaneous disorder commonly caused by drugs. TEN is often treated with corticosteroids, intravenous immunoglobulin (IVIG), or cyclosporine; however, the efficacy of these treatments is controversial. Etanercept (a TNF-α antagonist) was proven to decrease skin-healing time in a randomized clinical trial. Herein, we report the case of a 44-month-old boy who developed TEN due to deflazacort as the probable culprit drug and was successfully treated with etanercept. The patient presented to the emergency department complaining of erythematous maculopapular rashes and vesicles all over the face and body, with vesicles on the hands, feet, and trunk. Symptoms started 4 days before presentation, with edema of the upper lip, which progressed to erythematous macules over the body. He was started on deflazacort for nephrotic syndrome 21 days before the visit. Approximately 20% of the body surface area (BSA) was covered by vesicular lesions. Under the diagnosis of Steven Johnson syndrome/TEN, deflazacort was discontinued, and intravenous dexamethasone (1.5 mg/kg/day), a 5-day course of IVIG (0.4 mg/kg/day), and cyclosporine (3 mg/kg/day) were administered. The lesions seemed to be stationary for 3 days, but on the 6th day of hospitalization, when IVIG was discontinued, the vesicular lesions progressed to approximately 60% of the BSA. Etanercept 0.8 mg/kg was administered subcutaneously. Lesions stopped progressing, and bullous lesions started epithelialization. However, on the 15th day, around 30% of the BSA was still involved; thus, a second dose of etanercept was administered. No acute or sub-acute complications were observed. In conclusion, the use of etanercept in children with TEN that is not controlled with conventional therapy is both effective and safe.

Paracetamol is considered to be a relatively safe drug, even in the pediatric age group, at the recommended doses. Here we present a case of a six-year-old male presenting with symptoms and signs of Steven Johnson syndrome/toxic epidermal necrosis (SJS/TEN) following the ingestion of paracetamol. Steven Johnson syndrome/toxic epidermal necrosis is a potentially life-threatening dermatological emergency requiring intensive treatment. The patient was initially misdiagnosed as a case of chickenpox and was administered paracetamol. However, upon attending a tertiary care facility, he was diagnosed with TEN and treated with immunosuppressants. He recovered fully without any complications and was discharged within a week.

暂无摘要。

Methazolamide is used to treat patients with glaucoma. However, as a sulfonamide derivative, methazolamide shares the same adverse reaction profile as other sulfa-based medications. Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare delayed-type hypersensitivity cutaneous reactions with high morbidity and mortality. Here, we report a severe SJS/TEN overlap syndrome in an 85-year-old Chinese male patient who received methazolamide 25 mg twice daily for his left eye glaucoma. The causal relationship between SJS/TEN and methazolamide was categorized as \"highly likely\" on the algorithm for assessing drug causality for epidermal necrolysis. In addition to the treatments with methylprednisolone and immunoglobulin, we used a special electromagnetic spectrum therapeutic apparatus to provide skin wound care. The patient had a thoroughly satisfying recovery. This is the first case report to use electromagnetic field therapy in a patient with SJS/TEN. We share our experience here and suggest that electromagnetic field therapy can provide advanced skin wound care and facilitate the recovery of SJS/TEN.

暂无摘要。

We aimed describe the chronic ocular sequelae of Kindler syndrome. All cases of Kindler syndrome with ocular involvement that presented to a tertiary eye care center were included. Three cases of Kindler syndrome with ocular changes were reviewed. Case 1 (10 years, female) had recurrent epithelial breakdown with severe dry eye and corneal opacity secondary to keratitis. Case 2 (28 years, male) had symblepharon , ocular surface keratinization , and severe dry eye. Case 3 (16 years , female ) had partial limbal stem cell deficiency with dry eye. All cases were treated with topical lubricants, short course of low-potency steroids and immuno-modulators. Attention must be paid to the eye in addition to the oro-an-genital mucosa to avoid longterm ocular sequelae.

BACKGROUND: The aim of this study was to describe the epidemiological, clinical and etiological aspects of severe cutaneous adverse drug reactions in children in dermatologyvenereology unit at National and Teaching Hospital of Cotonou.
METHODS: A retrospective and descriptive study was carried out for 10 years in dermatology-venereology unit at the National and Teaching Hospital of Cotonou to document the epidemiological, clinical and etiological aspects of severe cutaneous adverse drug reactions in children. It included all children aged from 0 to 18 years with clinical diagnosis of severe cutaneous adverse drug reactions. Drug imputability was based on the criteria of the French pharmacovigilance group.
RESULTS: Severe cutaneous adverse drug reactions accounted for 47.3% of paediatric cases (35/74 cases). The mean age was 9.3 years ± 5.2. The sex-ratio was 1.1. Self-medication was noted in 76.5% of children, on the initiative of parents in 66.7% of cases. There were 51.4% cases of Steven Johnson syndrome, 22.8% cases of Lyell syndrome, 8.5% cases of generalized and bullous fixed drug eruption, 2.9% cases of acute generalized exanthematous pustulosis and erythrodermic maculo-papular rash. Drug combinations was noted in 20% of cases. Penicillins (26.5%), paracetamol and sulfonamides (16.3%) were the drugs frequently incriminated.
CONCLUSIONS: Steven Johnson syndrome and Lyell syndrome were the main severe cutaneous adverse drug reactions in children, mostly of school age. Penicillins, paracetamol and sulfonamides were the drugs frequently used and administered most often on self-medication.
BACKGROUND: L’objectif de cette étude était de décrire les aspects épidémiologiques, cliniques et étiologiques des toxidermies graves chez les enfants en dermatologie à Cotonou.
METHODS: Une étude rétrospective et descriptive a été réalisée sur 10 ans dans le service de dermatologie du Centre National Hospitalier et Universitaire de Cotonou pour documenter les aspects épidémiologiques, cliniques et étiologiques des toxidermies graves chez les enfants. Étaient inclus tous les enfants âgés de 0-18 ans chez qui le diagnostic clinique de toxidermie grave a été retenu. L’imputabilité médicamenteuse était basée sur les critères du groupe français de pharmacovigilance.
RESULTS: Les toxidermies graves représentaient 47,3% des cas pédiatriques (35/74 cas). L’âge moyen était de 9,3 ans ± 5,2. La sex-ratio H/F était de 1,1. Une automédication a été notée chez 76,5% des enfants, sur l’initiative des parents dans 66,7% des cas. Il y avait 51,4% de cas de syndrome de Steven Johnson, 22,8% de cas de syndrome de Lyell, 8,5% de cas d’érythème pigmenté fixe bulleux étendu, 2,9% de pustulose exanthématique aigüe généralisée et d’exanthème maculo-papuleux eìrythrodermique. Une polymédication a été notée dans 20% des cas. Les pénicillines (26,5%), le paracétamol et les sulfamides (16,3%) étaient les médicaments fréquemment incriminés.
CONCLUSIONS: Le syndrome de Steven Johnson et le syndrome de Lyell étaient les principales toxidermies graves chez les enfants, majoritairement en âge scolaire. Les pénicillines, le paracétamol et les sulfamides étaient les médicaments fréquemment incriminés et administrés le plus souvent en automédication.
UNASSIGNED: Toxidermies graves, syndrome de Steven Johnson, syndrome de Lyell, automédication, sulfamides, enfants, Benin.