Treatment of Stevens-Johnson Syndrome

Initial Management

The British Journal of Dermatology recommends immediate discontinuation of any potential culprit drug as the first and most critical step in SJS management 1
Assess severity using SCORTEN to predict mortality risk 2
Transfer to a specialized care center with experience managing SJS/TEN, preferably a burn unit or ICU for patients with >10% body surface area epidermal detachment 3, 4
Barrier nursing in a temperature-controlled room on a pressure-relieving mattress 3

Supportive Care

Fluid Management

Careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding fluid overload that can lead to pulmonary, cutaneous, and intestinal edema 2
Monitor fluid balance with regular assessment of vital signs, urine output, and electrolytes 1

Wound Care

Gentle skin handling to minimize shearing forces and prevent further epidermal detachment 3
Frequent application of bland emollients to support barrier function and reduce transcutaneous water loss 2
Appropriate dressings on exposed dermis to reduce fluid and protein loss, limit microbial colonization, control pain, and accelerate re-epithelialization 2
Consider petrolatum emollients or dimethicone as alternatives for skin protection 5, 6
High-strength topical corticosteroids may be used on affected skin areas 5, 6

Infection Prevention and Management

Monitor for signs of infection rather than using prophylactic antibiotics, which may increase skin colonization 2
Regular skin swabs for culture to detect predominant organisms 2
Targeted antimicrobial therapy only when clinical signs of infection are present 2

Mucosal Management

Ocular Care

Early ophthalmology consultation for all patients with ocular involvement 5, 6
Frequent lubrication with preservative-free artificial tears and lubricating ointments 7
Daily examination for symblepharon formation 7
Lysis of adhesions performed by an ophthalmologist to prevent permanent scarring 7
Topical antibiotics when corneal fluorescein staining or ulceration is present 7, 8
Topical corticosteroid drops under ophthalmologist supervision may reduce ocular surface damage 8

Oral Care

Anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating 9
Antiseptic oral rinse twice daily 9
Topical anesthetics such as viscous lidocaine 2% or cocaine mouthwashes 2-5% for severe oral discomfort 9
Monitor for and treat secondary infections with appropriate antifungals or antivirals if HSV is suspected 9
Topical corticosteroids may be applied to oral mucosa 9

Urogenital Care

Urinary catheterization when urogenital involvement causes dysuria or retention, or to monitor output 1
Regular examination of urogenital tract during acute illness 9
White soft paraffin ointment applied to urogenital skin and mucosae every 4 hours 9
Consider vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation 9

Systemic Therapy Options

Systemic corticosteroids, such as IV methylprednisolone, may be beneficial if started early, preferably within 72 hours 5, 6

Special Considerations

A multidisciplinary approach involving dermatology, intensive care, ophthalmology, and specialist skincare nursing is essential 3
Additional specialists may be needed based on organ involvement 3

Common Pitfalls to Avoid

Delayed recognition and treatment significantly increases mortality risk 4
Indiscriminate use of prophylactic antibiotics may increase skin colonization with resistant organisms 2
Overaggressive fluid resuscitation can lead to pulmonary, cutaneous, and intestinal edema 2
Failure to involve appropriate specialists early, particularly ophthalmology, can lead to permanent sequelae 7
Continued use of the culprit medication will worsen the condition and increase mortality 1

Management of Stevens-Johnson Syndrome

Supportive Care

The British Journal of Dermatology recommends delivering continuous enteral nutrition throughout the acute phase, providing 20-25 kcal/kg daily during the catabolic phase and 25-30 kcal/kg during recovery, and consider nasogastric feeding when oral intake is precluded by buccal mucositis 10
The British Journal of Dermatology suggests regularly cleansing wounds by gently irrigating with warmed sterile water, saline, or chlorhexidine (1/5000), and applying greasy emollient over the entire epidermis, including denuded areas 10
The British Journal of Dermatology recommends leaving detached epidermis in situ to act as a biological dressing, and decompressing blisters by piercing and expression or aspiration of fluid 10
The British Journal of Dermatology advises applying nonadherent dressings to denuded dermis with secondary foam or burn dressings to collect exudate, and considering silver-containing products/dressings for sloughy areas only 10
The British Journal of Dermatology recommends providing adequate background simple analgesia with additional opioid analgesia for breakthrough pain 10

Mucosal Management

The British Journal of Dermatology suggests arranging ophthalmological examination within 24 hours of diagnosis and continuing daily reviews during the acute illness, and applying preservative-free lubricant eye drops every two hours throughout the acute illness 11
The British Journal of Dermatology recommends performing daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions 11
The British Journal of Dermatology advises using topical antibiotics when corneal fluorescein staining or ulceration is present, and considering topical corticosteroids for ocular inflammation under ophthalmologist supervision 11

Systemic Therapy Options

The British Journal of Dermatology recommends ciclosporin (3 mg/kg daily for 10 days, tapered over 1 month) as it has shown benefit in multiple studies with reduced mortality compared to predicted rates 12
The British Journal of Dermatology suggests systemic corticosteroids, particularly early methylprednisolone pulse therapy, may be beneficial if started within 72 hours of onset 13

Discharge Planning and Follow-up

The British Journal of Dermatology recommends providing written information about the culprit drug(s) to avoid and any potentially cross-reactive medications, and encouraging patients to wear a MedicAlert bracelet or amulet bearing the name of the culprit drug 12
The British Journal of Dermatology advises documenting the drug allergy in the patient's medical records and informing all healthcare providers involved in their care, and reporting the adverse drug reaction to pharmacovigilance authorities 13
The British Journal of Dermatology suggests informing patients about potential fatigue and lethargy for several weeks following discharge and the need for convalescence, and considering referral to support groups such as SJS Awareness U.K. 12

Treatment of Stevens-Johnson Syndrome

Initial Management

The British Journal of Dermatology recommends assessing severity using SCORTEN to predict mortality risk, and transferring patients with >10% body surface area epidermal detachment to a burn center or ICU with experience in treating SJS/TEN 14, 15
Patients should be barrier-nursed in a temperature-controlled room (25-28°C) on a pressure-relieving mattress 14

Supportive Care

Skin Management

Minimize shearing forces when handling skin to prevent further epidermal detachment, and apply bland emollients frequently to support barrier function and reduce water loss 14
Use appropriate dressings on exposed dermis to reduce fluid/protein loss, limit microbial colonization, control pain, and accelerate re-epithelialization 14

Infection Prevention

Monitor for signs of infection rather than using prophylactic antibiotics, which may increase skin colonization, and obtain regular skin swabs for culture to detect predominant organisms 14, 15
Use targeted antimicrobial therapy only when clinical signs of infection are present 14, 15

Multidisciplinary Approach

A multidisciplinary team approach is essential, involving dermatology, intensive care, specialist skincare nursing, and additional specialists based on organ involvement 14, 15

Common Pitfalls to Avoid

Indiscriminate use of prophylactic antibiotics may increase skin colonization with resistant organisms 14, 15

Management of Stevens-Johnson Syndrome

Initial Assessment and Triage

The British Journal of Dermatology recommends immediately discontinuing all potential culprit drugs and transferring patients to a specialized burn unit or ICU, particularly when body surface area involvement exceeds 10%, while initiating comprehensive supportive care and early systemic immunomodulation with either cyclosporine or corticosteroids within 72 hours of onset 16
Calculate SCORTEN on admission to predict mortality risk and guide intensity of care, as recommended by the British Journal of Dermatology 17, 18
Obtain skin biopsy to confirm diagnosis and exclude other blistering disorders, looking for confluent epidermal necrosis with subepidermal vesicle formation, as suggested by the British Journal of Dermatology 16
Document the exact date of rash onset and progression pattern, and record all medications taken in the previous 2 months, including over-the-counter and herbal products, with start dates, as recommended by the British Journal of Dermatology 16

Supportive Care

Use pressure-relieving mattress with barrier nursing techniques, as recommended by the British Journal of Dermatology 16
Monitor vital signs, urine output, and electrolytes regularly, as suggested by the British Journal of Dermatology 16

Mucosal Management

Ophthalmology consultation within 24 hours of diagnosis with daily examinations throughout acute phase, as recommended by the British Journal of Dermatology 17, 18

Quality Metrics for Audit

SCORTEN performed on admission, as recommended by the British Journal of Dermatology 17, 18
Ophthalmology consultation within 24 hours with daily assessments, as suggested by the British Journal of Dermatology 17, 18
GP notification at discharge with drug avoidance counseling and MedicAlert request, as recommended by the British Journal of Dermatology 17, 18

Treatment of Stevens-Johnson Syndrome

Immediate Actions

The British Journal of Dermatology recommends immediately discontinuing all suspected culprit drugs and transferring the patient to a specialized burn unit or ICU with experience managing SJS/TEN, particularly when body surface area involvement exceeds 10% 19, 20
Calculate SCORTEN on admission to predict mortality risk and guide intensity of care, as recommended by the British Journal of Dermatology 20
Transfer without delay to centers with multidisciplinary teams including dermatology, intensive care, burn surgery, and ophthalmology, as recommended by the British Journal of Dermatology 19, 20
Early transfer reduces mortality; delays in specialized care adversely affect outcomes, according to the British Journal of Dermatology 19

Supportive Care Framework

Infection Management

The British Journal of Dermatology recommends not using prophylactic antibiotics as they increase skin colonization with resistant organisms, particularly Candida 20
Institute targeted antimicrobial therapy only when clinical signs of infection appear, as recommended by the British Journal of Dermatology 20
Monitor carefully as fever from SJS/TEN itself complicates detection of secondary sepsis, according to the British Journal of Dermatology 20

Special Considerations for Pediatric Patients

Infection causes up to 50% of pediatric SJS/TEN cases, and the British Journal of Dermatology recommends testing for infective triggers and consulting infectious disease team in all pediatric cases 19
Manage pediatric patients in age-appropriate specialist units with pediatric intensivists and skin loss specialists, as recommended by the British Journal of Dermatology 19
High-risk children need quicker transfer to specialized care, according to the British Journal of Dermatology 19

Common Pitfalls to Avoid

Delayed recognition and transfer to specialized care significantly increases mortality, as reported by the British Journal of Dermatology 19
Indiscriminate prophylactic antibiotics increase resistant organism colonization, according to the British Journal of Dermatology 20

Management of Stevens-Johnson Syndrome

Mucosal Management

The British Journal of Dermatology recommends arranging ophthalmological examination within 24 hours of diagnosis with daily reviews during the acute illness, and applying preservative-free lubricant eye drops every 2 hours throughout the acute illness, to prevent permanent ocular sequelae 21
The British Journal of Dermatology suggests performing daily ocular hygiene by an ophthalmologist or ophthalmically trained nurse to remove inflammatory debris and break down conjunctival adhesions, and considering amniotic membrane transplantation (AMT) in the acute phase to demonstrate significantly better visual outcomes compared to medical management alone 21
The British Journal of Dermatology recommends examining the mouth as part of initial assessment with daily oral review during acute illness, and applying white soft paraffin ointment to the lips immediately, then every 2 hours throughout the acute illness, to reduce risk of fibrotic scars 21
The British Journal of Dermatology suggests using anti-inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, particularly before eating, and antiseptic oral rinse twice daily to reduce bacterial colonization 21
The British Journal of Dermatology recommends treating candidal infection with nystatin oral suspension 100,000 units four times daily for 1 week, or miconazole 21

Management of Stevens-Johnson Syndrome

Initial Assessment and Treatment

The British Journal of Dermatology recommends immediately discontinuing all suspected culprit drugs and transferring the patient to a specialized burn unit or ICU with multidisciplinary expertise, particularly when body surface area involvement exceeds 10%, while initiating comprehensive supportive care as the cornerstone of management 22, 23
Calculate SCORTEN on admission to predict mortality risk and guide intensity of care, as recommended by the British Journal of Dermatology 23
Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over the entire epidermis including denuded areas every few hours, as suggested by the British Journal of Dermatology 22, 23

Supportive Care

Provide careful fluid resuscitation to prevent end-organ hypoperfusion while avoiding overaggressive replacement that leads to pulmonary, cutaneous, and intestinal edema, as recommended by the British Journal of Dermatology 23
Minimize shearing forces when handling skin to prevent further epidermal detachment, as suggested by the British Journal of Dermatology 23
Leave detached epidermis in situ to act as a biological dressing, as recommended by the British Journal of Dermatology 23

Infection Prevention and Management

Do not use prophylactic antibiotics, as indiscriminate administration increases skin colonization with resistant organisms, particularly Candida albicans, as recommended by the British Journal of Dermatology 22, 23
Monitor for rising C-reactive protein and neutrophilia as indicators of sepsis, as suggested by the British Journal of Dermatology 23

Mucosal Management

Arrange ophthalmological examination within 24 hours of diagnosis with daily reviews throughout the acute illness, as recommended by the British Journal of Dermatology 22
Apply preservative-free lubricant eye drops every 2 hours throughout the acute illness, as suggested by the British Journal of Dermatology 22
Perform daily oral review during the acute illness, as recommended by the British Journal of Dermatology 22
Apply white soft paraffin ointment to the lips every 2 hours, as suggested by the British Journal of Dermatology 22

Systemic Immunomodulatory Therapy

IVIg has been used but evidence is equivocal—pooled analysis showed no mortality benefit (OR 1.00, 95% CI 0.58-1.75), as reported by the British Journal of Dermatology 24
Thalidomide (an anti-TNF agent) was associated with excess deaths in one randomized trial and should be avoided, as reported by the British Journal of Dermatology 24

Airway Management

Respiratory symptoms and hypoxemia on admission should prompt urgent discussion with an intensivist and rapid transfer to ICU or burn center, as recommended by the British Journal of Dermatology 22, 24
Perform fibreoptic bronchoscopy to identify bronchial involvement, evaluate prognosis, investigate pneumonitis, and mechanically remove sloughed bronchial epithelium, as suggested by the British Journal of Dermatology 22, 24

Discharge Planning and Follow-up

Encourage patients to wear a MedicAlert bracelet bearing the name of the culprit drug, as recommended by the British Journal of Dermatology 22
Document drug allergy in the patient's medical records and inform all healthcare providers involved in their care, as suggested by the British Journal of Dermatology 22
Report the adverse drug reaction to national pharmacovigilance authorities, as recommended by the British Journal of Dermatology 22

Treatment of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Immediate Life-Saving Actions

The British Journal of Dermatology recommends immediately discontinuing all suspected culprit medications and transferring patients with >10% body surface area (BSA) epidermal detachment to a specialized burn center or ICU within hours of diagnosis, as delayed transfer significantly increases mortality risk 25, 26
The British Journal of Dermatology suggests calculating SCORTEN within the first 24 hours to predict mortality, with scores ranging from 0-7 and mortality ranging from 1% to 99% 26

Specialized Care Environment

Patients must be barrier-nursed in a temperature-controlled side room on a pressure-relieving mattress with humidity control, as recommended by the British Journal of Dermatology, to prevent hypothermia and reduce infection risk 25, 26

Supportive Care Framework

Fluid Management

The British Journal of Dermatology recommends establishing adequate intravenous fluid replacement guided by urine output and hemodynamic parameters 25
Monitor fluid balance carefully with urinary catheterization when indicated, as suggested by the British Journal of Dermatology 25

Wound Care Protocol

Handle skin with extreme care to minimize shearing forces that cause further epidermal detachment, as recommended by the British Journal of Dermatology 26
Irrigate wounds gently using warmed sterile water, saline, or chlorhexidine, as suggested by the British Journal of Dermatology 25
Apply greasy emollient over entire epidermis including denuded areas every few hours, as recommended by the British Journal of Dermatology 25
Leave detached epidermis in situ to act as biological dressing, as suggested by the British Journal of Dermatology 25

Infection Prevention and Management

The British Journal of Dermatology recommends against using prophylactic systemic antibiotics, as this increases skin colonization with resistant organisms 25
Take swabs for bacterial and candidal culture from three lesional areas on alternate days, as suggested by the British Journal of Dermatology 25
Administer systemic antibiotics only when clinical signs of infection appear, as recommended by the British Journal of Dermatology 25

Nutrition Support

Provide continuous enteral nutrition throughout the acute phase, as suggested by the British Journal of Dermatology 25
Deliver 20-25 kcal/kg daily during the catabolic phase, as recommended by the British Journal of Dermatology 25
Increase to 25-30 kcal/kg daily during the anabolic recovery phase, as suggested by the British Journal of Dermatology 25

Pain Management

Use validated pain assessment tools at least once daily, as recommended by the British Journal of Dermatology 25
Provide adequate background analgesia to ensure comfort at rest, as suggested by the British Journal of Dermatology 25, 27

Additional Supportive Measures

Administer low molecular weight heparin as prophylactic anticoagulation for immobile patients, as recommended by the British Journal of Dermatology 27
Provide proton pump inhibitor if enteral nutrition cannot be established, as suggested by the British Journal of Dermatology 27
Consider recombinant human G-CSF for neutropenic patients, as recommended by the British Journal of Dermatology 27

Multidisciplinary Team Coordination

Care must be coordinated by a specialist in skin failure with a team including intensive care physicians, ophthalmologists, and specialist skincare nurses, as recommended by the British Journal of Dermatology 26

Management of Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)

Clinical Examination Approach

The British Journal of Dermatology recommends documenting the exact date of rash onset and progression pattern over hours to days, as well as recording all vital signs including oxygen saturation with pulse oximetry, baseline body weight, and assessing airway patency 28
The British Journal of Dermatology suggests examining the skin for purpuric macules, flat atypical targets, flaccid blisters, and areas of epidermal detachment, and recording the extent of erythema and epidermal detachment separately on a body map using Lund and Browder chart 28
The British Journal of Dermatology recommends examining the mouth, eyes, and genitalia for erosive and hemorrhagic mucositis, blisters, and erosions, and considering the involvement of mucous membranes as an early diagnostic hallmark 28
The British Journal of Dermatology suggests taking photographs of the skin for documentation and monitoring progression 28

Investigations

The British Journal of Dermatology recommends performing full blood count, C-reactive protein, urea and electrolytes, liver function tests, coagulation studies, glucose, magnesium, phosphate, bicarbonate, base excess, and lactate as mandatory initial laboratory tests 28
The British Journal of Dermatology suggests performing mycoplasma and chlamydia serology, particularly in children and young adults, as part of the infectious disease workup 28
The British Journal of Dermatology recommends performing skin swabs for HSV and varicella zoster virus PCR, and considering chest X-ray if respiratory symptoms are present 28

Special Considerations for Pediatric Patients

The British Journal of Dermatology suggests testing for infective triggers and consulting infectious disease team in all pediatric cases, and considering targeted antibiotics as appropriate (e.g., azithromycin for mycoplasma) 28

Outcome Measures for Monitoring

The British Journal of Dermatology recommends monitoring critical outcomes such as survivorship/survival, internal organ dysfunction, no residual impairment, and quality of life/psychosocial well-being 28

Drug History Documentation

The British Journal of Dermatology suggests recording all medicines taken and vaccinations received over the preceding 2 months, including over-the-counter and complementary/alternative therapies, and using ALDEN (ALgorithm of Drug causality in Epidermal Necrolysis) online tool to predict likely causality 28

High-Risk Patients

The British Journal of Dermatology recommends identifying patients with likely drug trigger and underlying diseases associated with worse prognosis (malignancy, previous stem cell transplant) 28

Ophthalmological Management

The British Journal of Dermatology suggests arranging examination by ophthalmologist experienced in ocular surface diseases within 24 hours of diagnosis, and continuing daily ophthalmological reviews throughout acute illness 28

Drug Causality and Immediate Discontinuation in Oseltamivir‑Associated Stevens‑Johnson Syndrome

Identification of the Causative Agent

In patients who develop Stevens‑Johnson syndrome shortly after starting oseltamivir, the temporal relationship strongly implicates the drug as the most likely trigger, even though oseltamivir is listed as a very rare cause of SJS in existing guidelines; therefore, immediate cessation of oseltamivir is recommended. The evidence is based on case‑report observations linking drug exposure to onset of rash. 29

Pharmacologic Interventions for Stevens‑Johnson Syndrome / Toxic Epidermal Necrolysis (Evidence 30)

Wound‑Care Pharmacology

High‑strength topical corticosteroids may be applied to affected skin areas as part of the wound‑care regimen. 30

Analgesia

Background analgesia should consist of simple (non‑opioid) analgesics, with additional opioid analgesia reserved for breakthrough pain episodes. 30

Systemic Immunomodulatory Therapy

Intravenous methylprednisolone at 0.5–1 mg/kg (or an equivalent corticosteroid) initiated within 72 hours of disease onset, followed by conversion to oral corticosteroids with a taper lasting at least 4 weeks, is recommended for early treatment. 30
For immune‑checkpoint‑inhibitor‑induced SJS/TEN:
- Grade 3 disease – use methylprednisolone 0.5–1 mg/kg. 30
- Grade 4 disease – use methylprednisolone 1–2 mg/kg. 30
IVIG or cyclosporine may be considered in patients with severe disease or those who do not respond adequately to corticosteroids. 30

Management of Immune‑Checkpoint‑Inhibitor–Induced SJS/TEN

Permanent discontinuation of the offending immune‑checkpoint inhibitor is required. 30
Unlike classic SJS/TEN, corticosteroids are not contraindicated in this setting because the pathology is driven by T‑cell–mediated immune toxicity; appropriate immune suppression is advised. 30
Patients should be admitted immediately to a burn unit or intensive‑care setting with dermatology and wound‑care consultation. 30
Pain or palliative‑care consultation should be offered for patients who present with overlapping DRESS (Drug Reaction with Eosinophilia and Systemic Symptoms) features. 30

Guidelines for Management of Stevens‑Johnson Syndrome / Toxic Epidermal Necrolysis

Initial Assessment and Early Intervention

Discontinue all suspected culprit medications immediately and transfer patients with >10 % body‑surface‑area epidermal detachment to a specialized burn centre or intensive‑care unit within hours of diagnosis, because delayed transfer markedly raises mortality risk. 31, 32
Calculate the SCORTEN score within the first 24 hours; scores from 0 to 7 correspond to predicted mortality rates ranging from 1 % to 99 %. 31
Arrange a mandatory ophthalmology consultation within 24 hours of diagnosis; failure to do so is associated with permanent visual impairment. 31, 32

Specialized Care Environment

Provide barrier nursing in a temperature‑controlled room (25–28 °C) on a pressure‑relieving mattress to prevent hypothermia and lower infection risk. 31

Skin and Wound Management

Handle the skin with extreme gentleness to avoid shearing forces that can cause further epidermal loss. 31
Cleanse wounds gently using warmed sterile water, saline, or dilute chlorhexidine (1 : 5000). 31
Deliver daily wound care by specialist nurses experienced with fragile skin disorders. 31
Apply a greasy emollient (50 % white soft paraffin + 50 % liquid paraffin) over the entire epidermis, including denuded areas, every 2–4 hours. 31
Leave detached epidermis in situ to serve as a biological dressing. 31
Reserve surgical debridement with biosynthetic xenograft or allograft for cases where conservative management fails (e.g., clinical deterioration, expanding detachment, local sepsis, delayed healing, or wound conversion). 31

Infection Prevention and Management

Do not give prophylactic systemic antibiotics; indiscriminate use promotes colonisation with resistant organisms, especially Candida spp. 31
Monitor for clinical infection signs (confusion, hypotension, oliguria, desaturation, increased skin pain, rising CRP, neutrophilia). 31
Initiate targeted antimicrobial therapy only when such clinical signs are present. 31

Ophthalmologic and Mucosal Care

Instill preservative‑free lubricating eye drops every 2 hours throughout the acute phase. 31
Use topical antibiotics when corneal fluorescein staining or ulceration is evident. 31
Under ophthalmology supervision, apply topical corticosteroid drops to mitigate ocular surface damage. 31
Rinse the oral cavity with a benzydamine‑containing anti‑inflammatory solution every 3 hours, especially before meals. 31
Perform twice‑daily antiseptic oral rinses to reduce bacterial colonisation. 31
Provide viscous lidocaine 2 % or cocaine mouthwash (2–5 %) for severe oral discomfort. 31
Employ urinary catheterisation when urogenital involvement causes dysuria, retention, or when accurate output monitoring is required. 31
Apply white‑soft‑paraffin ointment to urogenital skin and mucosa every 4 hours; consider vaginal dilators or tampons wrapped in Mepitel to prevent synechiae. 31

Fluid, Electrolyte, and Nutritional Support

Monitor fluid balance closely using vital signs, urine output, and electrolyte panels. 31
Provide continuous enteral nutrition delivering 20–25 kcal/kg/day during the catabolic phase, increasing to 25–30 kcal/kg/day during recovery. 31

Pediatric Considerations

Infectious triggers account for up to 50 % of pediatric SJS/TEN; test for Mycoplasma pneumoniae and herpes simplex virus and involve infectious‑disease specialists. 32
Mortality in children is lower than in adults (0–8.5 % depending on severity), but recurrence occurs in up to 18 % of cases. 32
Ensure ophthalmology assessment within 24 hours to avoid permanent visual sequelae, which are the most common long‑term complication in children. 32

Discharge Planning and Long‑Term Follow‑Up

Provide patients with written information about the identified culprit drug(s) and any cross‑reactive agents. 31
Advise patients to wear a MedicAlert bracelet naming the culprit drug. 31
Record the drug allergy in the medical record and communicate it to all healthcare providers. 31
Report the adverse drug reaction to national pharmacovigilance authorities. 31
Counsel patients that fatigue and lethargy may persist for several weeks after discharge. 31
Offer referral to support groups such as SJS Awareness U.K. 31

Critical Pitfalls to Avoid

Examining the patient without awareness of skin fragility can cause additional epidermal detachment; all clinicians must be warned of this risk. 31

Guidelines for Management of Stevens‑Johnson Syndrome and Toxic Epidermal Necrolysis

Initial Assessment and Triage

Calculate the SCORTEN score within the first 24 hours to predict mortality (scores 0‑7 correspond to 1‑99 % mortality) and guide intensity of care. 33, 34
Immediately discontinue all suspected culprit drugs and arrange transfer of patients with >10 % body‑surface‑area epidermal detachment to a specialized burn centre or ICU within hours of diagnosis, as delayed transfer markedly increases mortality. 33, 34

Specialized Care Environment

Admit patients to a burn centre or ICU staffed by a multidisciplinary team (dermatology, intensive care, ophthalmology, specialised nursing). 33
Provide barrier nursing in a temperature‑controlled room (25‑28 °C) on a pressure‑relieving mattress to prevent hypothermia and reduce infection risk. 33, 34
Early transfer to a specialised centre reduces mortality; any delay adversely affects outcomes. 33

Skin and Wound Management

Handling and Basic Care

Handle skin with extreme gentleness using antishear techniques to minimise further epidermal detachment. 33, 34
Daily bedside care should be performed by specialist nurses experienced with skin‑fragility disorders. 33, 34

Wound Cleansing and Dressing

Cleanse wounds gently by irrigating with warmed sterile water, saline, or dilute chlorhexidine (1 : 5000). 34
Apply a greasy emollient (50 % white soft paraffin + 50 % liquid paraffin) over the entire epidermis, including denuded areas, every 2‑4 hours to support barrier function, reduce trans‑epidermal water loss and promote re‑epithelialisation. 33, 34
Leave detached epidermis in situ to act as a biological dressing. 34
Decompress blisters by piercing and expressing or aspirating fluid. 34
Use non‑adherent dressings (e.g., Mepitel or Telfa) over denuded dermis, covered with secondary foam or burn dressings to collect exudate. 34
Apply silver‑containing dressings only to sloughy areas. 33, 34
Reserve surgical debridement with biosynthetic xenograft or allograft for patients with clinical deterioration, extension of detachment, local sepsis, delayed healing, or wound conversion. 33, 34

Infection Prevention and Management

Prophylactic systemic antibiotics should not be used, as they increase colonisation with resistant organisms (particularly Candida albicans) without improving outcomes. 33, 34
Obtain bacterial and candidal swabs from three lesional sites on alternate days for culture. 34
Monitor closely for infection signs: confusion, hypotension, oliguria, desaturation, increased skin pain, rising C‑reactive protein, and neutrophilia. 33, 34
Appearance of a monoculture of the same organism from multiple sites signals a predominant strain and raises the risk of invasive infection. 33, 34
Initiate systemic antibiotics only when clinical infection signs are present. 33, 34
Consider herpes simplex virus re‑activation in slowly healing eroded or vesicular areas, especially genital and oral sites. 34

Fluid, Electrolyte, and Nutritional Support

Avoid over‑aggressive fluid resuscitation, which can cause pulmonary, cutaneous, and intestinal oedema. 34
Monitor fluid balance regularly using vital signs, urine output, and electrolyte measurements. 33
Provide continuous enteral nutrition: 20‑25 kcal/kg/day during the catabolic phase, increasing to 25‑30 kcal/kg/day during recovery. 34
Use nasogastric feeding when oral intake is impossible due to buccal mucositis. 34

Mucosal Management

Ophthalmologic Care

Perform an ophthalmology examination within 24 hours of diagnosis and repeat daily throughout the acute phase. 33, 34
Apply preservative‑free lubricating eye drops every 2 hours during the acute illness. 34
Conduct daily ocular hygiene by an ophthalmologist or trained nurse to remove debris and break conjunctival adhesions. 34
Use topical antibiotics when corneal fluorescein staining or ulceration is present. 33, 34
Apply topical corticosteroid eye drops under ophthalmology supervision to reduce ocular surface damage. 33, 34

Oral Care

Apply white soft paraffin ointment to the lips immediately and then every 2 hours throughout the acute phase. 34
Use an anti‑inflammatory oral rinse containing benzydamine hydrochloride every 3 hours, especially before meals. 33, 34
Perform an antiseptic oral rinse twice daily to lower bacterial colonisation. 34
Provide topical anesthetic (viscous lidocaine 2 % or cocaine mouthwash 2‑5 %) for severe oral discomfort. 33
Apply topical corticosteroids to oral mucosa as needed. 33

Urogenital Care

Insert a urinary catheter when urogenital involvement causes dysuria, retention, or to monitor output. 33
Apply white soft paraffin ointment to urogenital skin and mucosa every 4 hours. 33
Use vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation. 33

Pain Management

Provide baseline non‑opioid analgesia to ensure comfort at rest. 34
Add opioid analgesia for breakthrough pain. 34

Systemic Immunomodulatory Therapy

Start intravenous methylprednisolone 0.5‑1 mg/kg (or equivalent) within 72 hours of disease onset, then switch to oral corticosteroids with a taper lasting at least 4 weeks. 33
Early methylprednisolone pulse therapy may be beneficial if initiated within 72 hours. 33, 34
Administer cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month; studies show reduced mortality compared with predicted rates. 34

Discharge Planning and Follow‑Up

Provide written information about the culprit drug(s) and any cross‑reactive medications to avoid. 34
Advise patients to wear a MedicAlert bracelet naming the culprit drug. 34
Document the drug allergy in the medical record and inform all healthcare providers. 34
Report the adverse drug reaction to national pharmacovigilance authorities. 34
Inform patients that fatigue and lethargy may persist for several weeks after discharge and that convalescence is required. 34
Offer referral to support groups such as SJS Awareness U.K. 34

Critical Pitfalls to Avoid

Delayed transfer to specialised care significantly increases mortality. 33
Indiscriminate prophylactic antibiotics increase resistant organism colonisation and do not improve outcomes. 33, 34
Continued use of the culprit medication worsens the condition and raises mortality. 33
Over‑aggressive fluid resuscitation leads to pulmonary, cutaneous, and intestinal oedema. 34

Systemic Immunomodulatory and Symptomatic Therapies for Stevens‑Johnson Syndrome

Topical Corticosteroid Use

Analgesic Management

Early Systemic Corticosteroid Therapy

Immune Checkpoint Inhibitor–Induced SJS/TEN

Drug Discontinuation

Corticosteroid Dosing by Severity

Contraindication Clarification

Acute Management of Stevens‑Johnson Syndrome: Evidence‑Based Recommendations

Immediate Life‑Saving Actions

Discontinue all suspected culprit drugs immediately and transfer patients with >10 % body‑surface‑area epidermal detachment to a specialized burn centre or intensive‑care unit within hours; delayed transfer markedly raises mortality. 36
Calculate the SCORTEN score within the first 24 h to estimate mortality risk (scores 0‑7 correspond to ≈ 1‑99 % mortality) and to guide intensity of care. 37
Arrange a mandatory ophthalmology consultation within 24 h of diagnosis; lack of early eye assessment is associated with permanent visual impairment. 37
Record every medication taken in the preceding two months, including over‑the‑counter products, with exact start dates. 38

Specialized Care Environment

Admit patients to a burn centre or ICU staffed by a multidisciplinary team (dermatology, intensive‑care, ophthalmology, specialist nursing). 37
Provide barrier nursing in a temperature‑controlled room (25‑28 °C) on a pressure‑relieving mattress to prevent hypothermia and lower infection risk. 37
Ensure daily bedside care is performed by nurses experienced in skin‑fragility disorders. 37

Skin and Wound Management

Handle skin with extreme gentleness using anti‑shear techniques to avoid further epidermal loss. 37
Clean wounds gently with warmed sterile water, saline, or dilute chlorhexidine (1:5000). 37
Apply a greasy emollient (50 % white soft paraffin + 50 % liquid paraffin) over the entire epidermis, including denuded areas, every 2‑4 h. 37
Leave detached epidermis in situ to serve as a biological dressing. 37
Decompress blisters by piercing and expressing or aspirating fluid. 37
Cover denuded dermis with non‑adherent dressings (e.g., Mepitel or Telfa) and a secondary foam or burn dressing to collect exudate. 37
Apply silver‑containing dressings only to sloughy areas. 37
Reserve surgical debridement with biosynthetic xenograft or allograft for clinical deterioration, extension of detachment, local sepsis, delayed healing, or wound conversion. 37

Infection Prevention and Management

Avoid prophylactic systemic antibiotics; they increase colonisation with resistant organisms (especially Candida spp.) without improving outcomes. 37
Obtain bacterial and candidal swabs from three lesional sites on alternate days for culture. 37
Monitor for infection signs (confusion, hypotension, oliguria, desaturation, increased skin pain, rising C‑reactive protein, neutrophilia). 37
Initiate targeted antimicrobial therapy only when clinical infection is evident. 37
Consider herpes simplex virus reactivation in slowly healing eroded or vesicular areas, particularly genital and oral sites. 37

Fluid, Electrolyte, and Nutritional Support

Avoid over‑aggressive fluid resuscitation, which can cause pulmonary, cutaneous, and intestinal oedema. 37
Monitor fluid balance regularly using vital signs, urine output, and electrolyte measurements. 37
Provide continuous enteral nutrition: 20‑25 kcal/kg/day during the catabolic phase, increasing to 25‑30 kcal/kg/day during recovery. 37
Use nasogastric feeding when oral intake is impossible due to buccal mucositis. 37

Mucosal Management

Ophthalmologic Care

Apply preservative‑free lubricating eye drops every 2 h throughout the acute phase. 37
Perform daily ocular hygiene by an ophthalmologist or trained nurse to remove debris and break conjunctival adhesions. 37
Use topical antibiotics when corneal fluorescein staining or ulceration is present. 37
Apply topical corticosteroid eye drops under ophthalmology supervision to reduce ocular surface damage. 37

Oral Care

Apply white soft paraffin ointment to the lips immediately and then every 2 h throughout the acute phase. 37
Use an anti‑inflammatory oral rinse containing benzydamine hydrochloride every 3 h, especially before meals. 37
Perform an antiseptic oral rinse twice daily to lower bacterial colonisation. 37
Provide a topical anesthetic (viscous lidocaine 2 % or cocaine mouthwash 2‑5 %) for severe oral discomfort. 37
Apply topical corticosteroids to oral mucosa as needed. 37

Urogenital Care

Insert a urinary catheter when urogenital involvement causes dysuria, retention, or when accurate output monitoring is required. 37
Apply white soft paraffin ointment to urogenital skin and mucosa every 4 h. 37
Use vaginal dilators or tampons wrapped in Mepitel to prevent vaginal synechiae formation. 37

Pain Management

Provide baseline non‑opioid analgesia for comfort at rest, adding opioid analgesia for breakthrough pain. 37

Systemic Immunomodulatory Therapy

Initiate intravenous methylprednisolone 0.5‑1 mg/kg (or equivalent) within 72 h of disease onset, then switch to oral corticosteroids with a taper of at least 4 weeks. 37
Consider cyclosporine 3 mg/kg daily for 10 days, tapered over 1 month; studies show reduced mortality compared with predicted rates. 37

Additional Supportive Measures

Administer low‑molecular‑weight heparin as prophylactic anticoagulation for immobile patients. 37
Provide a proton‑pump inhibitor when enteral nutrition cannot be established. 37

Discharge Planning and Follow‑Up

Give patients written information about the culprit drug(s) and any cross‑reactive medications to avoid. 36
Advise patients to wear a MedicAlert bracelet naming the culprit drug. 36
Document the drug allergy in the medical record and inform all healthcare providers. 36
Report the adverse drug reaction to national pharmacovigilance authorities. 36
Inform patients that fatigue and lethargy may persist for several weeks after discharge and that a period of convalescence is required. 36

Critical Pitfalls to Avoid

Delayed transfer to specialized care significantly increases mortality. 37
Failure to involve ophthalmology within 24 h leads to permanent visual impairment. 37

Evidence‑Based Practices for Stevens‑Johnson Syndrome Management

Risk Assessment

Calculate the SCORTEN score within the first 24 hours of admission to estimate mortality (scores 0–7 correspond to ≈ 1 %–99 % mortality) and to guide the intensity of care. 39

Medication History Documentation

Record every medication taken in the two months preceding onset—including prescription, over‑the‑counter, and herbal products—with exact start dates to aid identification of the offending agent. 39

Common Culprit Medications

Recognize that the most frequently implicated drugs are allopurinol, carbamazepine, lamotrigine, nevirapine, oxicam‑type NSAIDs, phenobarbital, phenytoin, sulfamethoxazole, and sulfasalazine. 39

Specialized Care Environment

Admit patients to a burn centre or intensive‑care unit that provides a multidisciplinary team (dermatology, intensive care, ophthalmology, and specialist skin‑care nursing). 39

Impact of Early Transfer

Transfer to a specialised centre as early as possible reduces mortality, whereas delayed transfer is associated with a significantly higher death rate. 39