What dermatologic emergencies exist and what is the recommended approach and management for each?

Dermatological Emergencies: Cases and Management

Dermatological emergencies requiring immediate recognition and intervention include Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis (SJS/TEN), erythroderma, acute generalized exanthematous pustulosis, drug reaction with eosinophilia and systemic symptoms (DRESS), pemphigus vulgaris, necrotizing fasciitis, and anaphylaxis with angioedema.

Major Dermatological Emergency: Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis

Recognition and Classification

SJS/TEN represents the most critical dermatological emergency with significant mortality risk. The spectrum is classified by extent of epidermal detachment 1:

• SJS: <10% body surface area (BSA) epidermal detachment plus widespread purpuric macules or flat atypical targets
• SJS/TEN overlap: 10-30% BSA detachment
• TEN: >30% BSA detachment

Initial Assessment and Triage

Immediately discontinue all suspected culprit drugs - most commonly allopurinol, carbamazepine, lamotrigine, nevirapine, oxicam NSAIDs, phenobarbital, phenytoin, sulfamethoxazole, and sulfasalazine 1.

Critical History Elements 1:

• Document medication history for 2 months prior to symptom onset (including over-the-counter preparations)
• Identify index date (first symptom: sore throat, rash, skin pain, sore eyes/mouth)
• Ask about respiratory symptoms (cough, dyspnea, hemoptysis)
• Ask about bowel involvement (diarrhea, abdominal distension)
• Previous herpes simplex virus infections

Physical Examination Priorities 1:

• Map extent of erythema AND epidermal detachment separately using Lund and Browder chart
• The detachment percentage (not erythema) has prognostic value
• Examine all mucosal sites (eyes, mouth, nose, genitalia)
• Look for atypical targets, purpuric macules, blisters
• Document vital signs and oxygen saturation

Prognostic Assessment: SCORTEN

Calculate SCORTEN within first 24 hours of admission 1. One point for each parameter present:

• Age >40 years
• Heart rate >120 bpm
• Malignancy
• BSA detachment >10%
• Serum urea >10 mmol/L
• Serum bicarbonate <20 mmol/L
• Serum glucose >14 mmol/L

Mortality prediction: 0-1 points = 1-4% mortality; 5 points = 85% mortality; 7 points = 99% mortality 1.

Immediate Management Algorithm

Step 1: Initial Investigations 1

• Full blood count, inflammatory markers, electrolytes (including magnesium, phosphate, bicarbonate)
• Liver function, coagulation studies
• Mycoplasma serology
• Chest X-ray
• Two skin biopsies: one for histopathology (lesional skin adjacent to blister), one for direct immunofluorescence (periblister skin, sent unfixed)
• Swabs from lesional skin for bacteriology
• Photograph skin involvement

Step 2: Resuscitation 1

• Establish IV access through non-lesional skin when possible
• Begin fluid resuscitation if clinically indicated
• Insert nasogastric tube if oral intake inadequate due to mucositis
• Insert urinary catheter when urogenital involvement present
• Initiate fluid balance chart

Transfer and Specialized Care

Patients with >10% BSA epidermal loss must be transferred without delay to a burn center or ICU with SJS/TEN experience 1. Delayed transfer is associated with increased mortality. Rapid admission to specialized centers improves survival 1.

Multidisciplinary Team Requirements 1:

Coordinated by dermatology/plastic surgery, including:

• Intensive care
• Ophthalmology (mandatory)
• Specialist skin care nursing
• Additional services: respiratory medicine, gastroenterology, gynecology, urology, oral medicine, microbiology, pain team, dietetics, physiotherapy, pharmacy

Specialized Unit Management

Environmental Control 1:

• Barrier nursing in side room with controlled humidity
• Pressure-relieving mattress
• Ambient temperature 25-28°C
• Minimize shearing forces during patient handling

Skin Management Regimen 1:

Conservative Approach (all patients):

• Irrigate wounds gently with warmed sterile water, saline, or chlorhexidine (1:5000)
• Apply greasy emollient (50% white soft paraffin with 50% liquid paraffin) over entire epidermis including denuded areas
• Apply topical antimicrobial to sloughy areas only (guided by microbiology; consider silver-containing products)
• Leave detached epidermis in situ as biological dressing
• Decompress blisters by piercing and aspirating fluid
• Apply non-adherent dressings (Mepitel™ or Telfa™) to denuded dermis
• Use secondary foam/burn dressing for exudate collection (Exu-Dry™)

Surgical Approach (for TEN >30% BSA with clinical deterioration) 1: Consider transfer to burn center for:

• Extension of epidermal detachment
• Subepidermal pus or local sepsis
• Wound conversion or delayed healing
• Debridement under general anesthesia
• Physiological closure with Biobrane/allograft/xenograft

Infection Control 1:

• Swab three areas of lesional skin on alternate days
• Administer systemic antibiotics ONLY if clinical signs of infection present
• Prophylactic antibiotics increase Candida colonization risk
• Monitor for sepsis: confusion, hypotension, reduced urine output, decreased oxygen saturation, rising C-reactive protein

Fluid Management 1:

• Site venous lines through non-lesional skin; change peripheral lines every 48 hours
• Catheterize and monitor fluid balance carefully
• Fluid requirements lower than burn formulas predict
• Estimated formula: body weight/% BSA epidermal detachment
• Avoid overaggressive resuscitation (causes pulmonary/cutaneous/intestinal edema)
• Progress to oral fluids as mouth involvement improves

Nutrition 1:

• Provide continuous enteral nutrition throughout acute phase
• Nasogastric feeding if buccal mucositis precludes oral intake
• Early catabolic phase: 20-25 kcal/kg/day
• Anabolic recovery phase: 25-30 kcal/kg/day
• Enteral preferred over parenteral to reduce peptic ulceration and bacterial translocation

Analgesia 1:

• Use validated pain assessment tool daily
• Provide adequate baseline analgesia for comfort at rest
• Supplementary opiates as required
• Additional analgesia for procedural pain during wound care

Pediatric Considerations

Children have lower mortality than adults but higher recurrence rates (up to 18%) 2. Infections are more common triggers than drugs in pediatric populations.

Pediatric-Specific Triage 2:

• Admit to pediatric ICU or burn center with dermatology support if:
  • BSA detachment >10%, OR
  • Comorbidity present, OR
  • Requiring ventilation
• Consider telemedicine consultation with specialist center
• Distinguish from erythema multiforme major (EMM), which has typical target lesions starting acrally, is infection-triggered, and does not progress to SJS/TEN

Other Critical Dermatological Emergencies

Erythroderma

Life-threatening condition with >90% BSA involvement requiring:

• Identification of underlying cause (psoriasis, eczema, drug reaction, cutaneous T-cell lymphoma)
• Temperature regulation
• High-output cardiac failure monitoring
• Fluid and protein replacement

Pemphigus Vulgaris

Autoimmune blistering disorder requiring:

• Immediate systemic immunosuppression
• Wound care similar to SJS/TEN
• Multidisciplinary approach

Necrotizing Fasciitis

Surgical emergency requiring:

• Immediate surgical debridement
• Broad-spectrum antibiotics
• Critical care support

Anaphylaxis with Angioedema

Requires:

• Immediate intramuscular epinephrine
• Airway assessment and management
• IV antihistamines and corticosteroids

Critical Pitfalls to Avoid

1. Delayed transfer: Transferring patients with >10% BSA involvement to specialized centers late increases mortality 1
2. Prophylactic antibiotics: Increases Candida colonization without benefit 1
3. Overaggressive fluid resuscitation: Use lower volumes than burn formulas predict 1
4. Misclassification: Distinguish SJS/TEN from erythema multiforme major, which has better prognosis 2
5. Inadequate ophthalmology involvement: Early and ongoing ophthalmology assessment mandatory to prevent blindness 1
6. Shearing forces: Minimize during patient handling to prevent further epidermal detachment 1