What are the guideline sources, guidelines and references for Stevens-Johnson syndrome ?
Guideline sources The following summarized guidelines for the evaluation and management of stevens-Johnson syndrome (SJS) are prepared by our editorial team based on guidelines from the Society of Dermatology Hospitalists (SDH 2020) and the British Association of Dermatologists (BAD 2019; 2016). 1 2 3 CalculatorSeverity of Illness Score for Toxi Guidelines 1. Classification and risk stratification https://web.pathway.md/diseases/recITRpKsnyyxBajU 1/6 6/24/23, 3:14 PM Stevens-Johnson syndrome Pathway Risk assessment: as per BAD 2016 guidelines, calculate the SCORTEN score within the first 24 hours of admission. B 2. Diagnostic investigations Initial assessment: as per BAD 2016 guidelines, elicit a detailed history in patients and/or relatives of patients with SJS. E Show 3 more Drug hypersensitivity testing: Do not obtain routine drug hypersensitivity testing following an episode of SJS/toxic epidermal necrolysis. D Seek specialist advice on hypersensitivity testing where: the culprit drug is not known medication avoidance is detrimental to the individual accidental exposure is possible. E Airway examination: as per BAD 2016 guidelines, perform fibreoptic bronchoscopy in patients with respiratory symptoms and hypoxemia on admission. E 3. Medical management Setting of care: as per BAD 2016 guidelines, ensure a multidisciplinary team coordinated by a specialist in skin failure, usually dermatology and/or plastic surgery, and including clinicians from intensive care, ophthalmology and skincare nursing. E Show 4 more Withdrawal of causative drug: as per BAD 2016 guidelines, identify and immediately withdraw causative agent. B Fluid management: as per BAD 2016 guidelines, establish peripheral venous access in patients with SJS. E Show 4 more Oral care: as per BAD 2016 guidelines, obtain daily oral review during the acute illness. E Show 5 more Ocular care, conservative management: as per BAD 2016 guidelines, obtain daily ophthalmological review during the acute illness. E Show 5 more Urogenital care: as per BAD 2016 guidelines, insert a urinary catheter if urogenital involvement is causing significant dysuria/retention. E Show 4 more Thromboprophylaxis: as per BAD 2016 guidelines, administer LMWH in immobile patients. B https://web.pathway.md/diseases/recITRpKsnyyxBajU 2/6 6/24/23, 3:14 PM Stevens-Johnson syndrome Pathway Antibiotics: as per BAD 2016 guidelines, administer systemic antibiotics only if there are clinical signs of infection. E Management of pain: as per BAD 2016 guidelines, use a patient-appropriate validated pain tool to assess pain in all conscious patients at least once daily. E Show 2 more Management of neutropenia: consider administering recombinant human G-CSF in patients with neutropenia. C 4. Nonpharmacologic interventions Enteral nutrition: as per BAD 2016 guidelines, insert a nasogastric tube and institute nasogastric feeding if the patient cannot maintain adequate nutrition PO. E Show 3 more 5. Surgical interventions Wound care: as per BAD 2016 guidelines, institute a conservative approach of wound care in all patients with SJS/toxic epidermal necrolysis. E Show 11 more 6. Specific circumstances Pediatric patients (differential diagnosis): exclude staphylococcal scalded skin syndrome by clinical assessment of mucosae (not involved in staphylococcal scalded skin syndrome) and skin biopsy if any diagnostic uncertainty. A Show 2 more Pediatric patients (risk assessment): Identify high-risk pediatric/young patients, such as patients with a likely drug trigger and underlying diseases associated with a worse prognosis (malignancy and previous stem cell transplant). A Consider calculating the SCORTEN score to provide a prognostic indicator. C Pediatric patients (history and physical examination): Elicit a detailed history in pediatric/young patients with SJS/toxic epidermal necrolysis and their parents/carers with specific reference to the following: Situation Guidance Fever, malaise, upper respiratory tract symptoms Prodromal illness Painful rash https://web.pathway.md/diseases/recITRpKsnyyxBajU 3/6 6/24/23, 3:14 PM Stevens-Johnson syndrome Pathway Onset (initially on the face and chest), date when the rash first appeared, and document the progression of the eruption Eyes, mouth, nose, and genitalia Mucosal involvement Pain, urinary retention Genital involvement Cough, dyspnea, bronchial hypersecretion, and hemoptysis Respiratory tract involvement Diarrhea, abdominal distension Bowel involvement When the patient developed the first symptom or sign of the disorder, such as sore throat, rash, skin pain, sore eyes/mouth Date Previous or ongoing medical problems, specifically history of previous drug reactions, recurrent HSV infections, chest infections, diagnosis and treatment for malignancy, and/or stem cell transplant. A Comorbidities Show 2 more Pediatric patients (laboratory tests): Obtain the following investigations in pediatric patients with SJS: CBC, CRP, urea and electrolytes, LFTs, coagulation studies, blood glucose, magnesium, phosphate, bicarbonate, base excess, lactate infection screening, as clinically relevant and following discussion with the infectious diseases team, such as mycoplasma and chlamydia serology, skin swabs for HSV and VZV, and CXR bacterial swabs from lesional skin for culture and sensitivity conjunctival swabs for bacteria, chlamydia, HSV (PCR), and adenovirus (PCR) photographs of the skin to show the type of lesion and extent of involvement skin biopsy from lesional skin, just adjacent to a blister, and send for routine histopathology; perform a second biopsy from perilesional skin and send unfixed for DIF, if required to exclude an immunobullous disorder; do not perform a biopsy if staphylococcal scalded skin syndrome is clinically typical, however, perform a shave biopsy of blister roof for a frozen section if staphylococcal scalded skin syndrome is considered but with diagnostic uncertainty. A Pediatric patients (setting of care): arrange a local multidisciplinary team led by a specialist in skin failure including dermatology and/or burns specialist, clinicians from pediatric intensive care, ophthalmology and pediatric tissue viability, and pediatric dermatology (if available) or experienced pediatric burns nurses. Consider ensuring additional clinical input to the multidisciplinary team from infectious diseases, respiratory medicine, hematology, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, play specialist, and pharmacy. Identify one specialist as the team coordinator. A https://web.pathway.md/diseases/recITRpKsnyyxBajU 4/6 6/24/23, 3:14 PM Stevens-Johnson syndrome Pathway Show 4 more Pediatric patients (withdrawal of causative drug): discontinue any potential culprit drug causing SJS/toxic epidermal necrolysis immediately. A Pediatric patients (initial stabilization): assess airways by a pediatric anesthesiologist or pediatric intensivist and consider performing intubation if clinical signs support this, especially if a transfer is planned. Ensure immediate availability of appropriate equipment for difficult intubation. A Consider involving an ear, nose, and throat team for further airway assessment. B Show 6 more Pediatric patients (fluid management): monitor fluid balance carefully and catheterize if clinically indicated. Establish adequate IV fluid replacement. Consider guiding fluid replacement by urine output and other end-point measurements. Adjust fluid replacement daily with careful monitoring of sodium levels. A Show 3 more Pediatric patients (nutritional support): provide nutrition early and throughout the acute phase, either by mouth or nasogastric/nasojejunal feeding if adequate oral intake is precluded by buccal mucositis. A Show 3 more Pediatric patients (management of pain): use an appropriate, validated tool to assess pain at least once daily in conscious patients. A Show 4 more Pediatric patients (immunomodulatory therapy): administer immunomodulatory therapy (such as IVIG), if instituted, under the supervision of a specialist skin failure multidisciplinary team in the context of clinical research and/or case registry. A Pediatric patients (skin care): ensure careful skin handling and reduce shearing forces to minimize the extent of epidermal detachment. B Show 21 more Pediatric patients (mouth care): obtain daily oral review during the acute phase. A Show 5 more Pediatric patients (eye care): obtain urgent ophthalmological assessment taking into account the extent of eyelid, conjunctival, and corneal involvement. A Show 7 more Pediatric patients (urogenital care): obtain daily urogenital review during the acute phase. A Show 5 more Pediatric patients (discharge and follow-up): provide written information and direct to available online support, such as patient support group. E Show 12 more 7. Patient education https://web.pathway.md/diseases/recITRpKsnyyxBajU 5/6 6/24/23, 3:14 PM Stevens-Johnson syndrome Pathway Counseling before discharge: Provide patients with a written information about drugs to avoid. E Encourage patients to wear a MedicAlert bracelet. E 8. Follow-up and surveillance Infection surveillance: as per BAD 2016 guidelines, employ strict barrier nursing to reduce nosocomial infections. E Follow-up: Arrange a dermatology outpatient clinic appointment, and, if required, an ophthalmology outpatient appointment, within a few weeks of discharge. E Refer for review to unit with appropriate subspeciality interest. E 9. Quality improvement Reporting: Document drug allergy in the patient's notes and inform all doctors involved in the patient's care. E Report the episode to the national pharmacovigilance authorities. E References 1. T McPherson, L S Exton, S Biswas et al. British Association of Dermatologists' guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in children and young people, 2018. Br J Dermatol. 2019 Jul;181 1 37 54. Open 2. Lucia Seminario-Vidal, Daniela Kroshinsky, Stephen J Malachowski et al. Society of Dermatology Hospitalists supportive care guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults. J Am Acad Dermatol. 2020 Jun;82 6 1553 1567. Open 3. D Creamer, S A Walsh, P Dziewulski et al. U.K. guidelines for the management of Stevens-Johnson syndrome/toxic epidermal necrolysis in adults 2016. Br J Dermatol. 2016 Jun;174 6 1194 227. Open 4. S Bastuji-Garin, N Fouchard, M Bertocchi et al. SCORTEN a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol. 2000 Aug;115 2 149 53. 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