Stevens-Johnson syndrome case study
The case
Patient: 27‑year‑old male, software engineer, no known chronic illnesses.
Presenting complaint: Sudden onset of painful, dusky‑red patches and blisters on the trunk and extremities for 3 days. He also reports fever and a burning sensation in his eyes for 2 days.
History of present illness:
- 7 days ago he started a course of lamotrigine 100 mg once daily for newly diagnosed focal epilepsy.
- 2 days after the first dose he noticed a mild sore throat and low‑grade fever (38.2 °C).
- On day 4 he developed a pruritic, erythematous maculopapular rash on his trunk, which progressed rapidly to targetoid lesions with central dusky necrosis.
- By day 5 the rash spread to the face, palms, soles and involved ~12 % of body surface area (BSA).
- He also reports oral mucosal erosions (painful swallowing), conjunctival injection with mild photophobia, and genital erosions.
- No recent travel, no known drug allergies, no similar past episodes.
Past medical history: Unremarkable except for a seizure disorder diagnosed 2 weeks ago.
Medication history: Lamotrigine (started 7 days ago), occasional ibuprofen for headache. No herbal or over‑the‑counter supplements.
Family history: Non‑contributory.
Social history: Non‑smoker, occasional alcohol, no illicit drug use.
Examination:
| Parameter | Finding |
|---|---|
| General | Ill‑appearing, febrile, in mild distress due to pain |
| Vitals | BP 118/72 mm Hg, HR 102 bpm, RR 18 /min, Temp 38.6 °C, SpO₂ 98 % on room air |
| Skin | Diffuse erythematous macules coalescing into target lesions with central dusky necrosis; positive Nikolsky sign on forearm; erosions on lips, buccal mucosa, genitalia; total BSA involvement ≈ 12 % (≈ > 10 % but < 30 %). |
| Eyes | Bilateral conjunctival injection, mild chemosis, no corneal ulceration. |
| ENT | Oropharyngeal erosions, mild dysphagia. |
| Lymph nodes | No palpable cervical or axillary lymphadenopathy. |
| Cardiovascular / Respiratory / Abdomen | Within normal limits. |
Initial investigations (values):
| Test | Result | Normal range |
|---|---|---|
| CBC | Hb 13.8 g/dL, WBC 12.5 × 10⁹/L (neutrophils 78 %), platelets 210 × 10⁹/L | Hb 13‑17 g/dL, WBC 4‑11 × 10⁹/L |
| CRP | 48 mg/L | < 5 mg/L |
| Serum electrolytes | Na⁺ 138 mmol/L, K⁺ 4.2 mmol/L, Cl⁻ 102 mmol/L, HCO₃⁻ 22 mmol/L | Na 135‑145 mmol/L, K 3.5‑5.0 mmol/L |
| Renal function | BUN 14 mg/dL, Creatinine 0.9 mg/dL | BUN 7‑20 mg/dL, Cr 0.6‑1.2 mg/dL |
| Liver function | AST 34 U/L, ALT 38 U/L, ALP 78 U/L, Bilirubin 0.9 mg/dL | AST/ALT < 40 U/L, ALP 30‑120 U/L, Bilirubin < 1.2 mg/dL |
| Blood cultures | No growth after 48 h | — |
| Serology for HSV‑1/2, Mycoplasma pneumoniae, HIV | Negative (ELISA) | — |
| Skin biopsy (H&E) | Full‑thickness epidermal necrosis with subepidermal bullae, sparse lymphocytic infiltrate, minimal eosinophils – consistent with SJS/TEN spectrum. | — |
| Urine output (first 24 h) | 1.8 mL/kg/h (≈ 130 mL/h) | ≥ 0.5 mL/kg/h (adequate) |
The patient is admitted to a high‑dependency unit (HDU) for close monitoring and multidisciplinary care.
Questions
- What is the most likely diagnosis?
- Which clinical and investigative findings support this diagnosis, and how do they differentiate it from other severe cutaneous adverse reactions?
- What are the key investigations that must be performed at admission and why?
- Outline the evidence‑based acute management plan, including pharmacologic and supportive measures, citing the latest guideline.
- What are the potential acute and long‑term complications of this condition, and what follow‑up is required?
Answers
1. What is the most likely diagnosis?
Stevens‑Johnson syndrome (SJS) – a severe mucocutaneous reaction characterized by epidermal necrosis involving < 10 % of body surface area (BSA) with mucosal involvement.
In this patient the BSA involvement is 12 %, which technically places him in the SJS/TEN overlap category (10‑30 % BSA). However, many examiners accept “SJS/TEN overlap” as the diagnosis when the BSA is just above 10 %. The key point is that the presentation belongs to the SJS/TEN spectrum of severe cutaneous adverse drug reactions (SCARs).
Why not other entities?
| Condition | Typical features | Reason for exclusion |
|---|---|---|
| Erythema multiforme (EM) minor | Target lesions limited to extremities, < 10 % BSA, no mucosal involvement | Our patient has extensive mucosal erosions and > 10 % BSA. |
| Drug reaction with eosinophilia and systemic symptoms (DRESS) | Facial edema, eosinophilia, organ involvement, latency 2‑6 weeks | Absence of eosinophilia, rapid onset (7 days), predominant skin necrosis. |
| Acute generalized exanthematous pustulosis (AGEP) | Sterile pustules, fever, neutrophilia, rapid resolution after drug withdrawal | No pustules; histology shows full‑thickness necrosis, not subcorneal pustules. |
| Toxic epidermal necrolysis (TEN) | > 30 % BSA involvement, massive epidermal detachment | BSA involvement is 12 %, below TEN threshold. |
Thus, the most appropriate label is SJS/TEN overlap (or simply SJS for exam simplicity).
2. Which clinical and investigative findings support this diagnosis, and how do they differentiate it from other severe cutaneous adverse reactions?
Key clinical clues
| Finding | Significance |
|---|---|
| Recent exposure to a high‑risk drug (lamotrigine) – onset 7 days after first dose. | Lamotrigine is a well‑documented trigger for SJS/TEN (incidence ≈ 0.1 %). |
| Prodromal flu‑like symptoms (fever, sore throat) preceding rash. | Typical in SJS/TEN; less common in EM. |
| Targetoid lesions with central dusky necrosis and positive Nikolsky sign. | Indicates epidermal necrosis; EM lesions are usually “target” without necrosis. |
| Mucosal involvement (oral, ocular, genital) affecting > 2 sites. | SJS/TEN requires ≥ 2 mucosal sites; DRESS may have limited mucosal disease. |
| BSA involvement 12 % – places patient in SJS/TEN overlap. | Distinguishes from EM (< 10 % BSA, no mucosa) and TEN (> 30 % BSA). |
| Rapid progression over 48‑72 h. | SJS/TEN evolves quickly; DRESS evolves over weeks. |
Investigative support
Skin biopsy – full‑thickness epidermal necrosis with subepidermal bullae, scant inflammatory infiltrate. This histology is classic for SJS/TEN and helps rule out EM (which shows interface dermatitis without full‑thickness necrosis).
CBC with neutrophilia, normal eosinophils – DRESS typically shows eosinophilia (> 700 cells/µL).
Negative serology for HSV, Mycoplasma – helps exclude infectious causes of EM.
Elevated CRP – reflects systemic inflammation but is non‑specific; however, it supports a severe drug reaction.
Overall, the combination of drug exposure, mucocutaneous findings, BSA involvement, histology, and laboratory profile points decisively toward SJS/TEN.
3. What are the key investigations that must be performed at admission and why?
| Investigation | Reason for ordering | Expected/critical value |
|---|---|---|
| Complete blood count (CBC) with differential | Detect leukocytosis, eosinophilia (DRESS), anemia, thrombocytopenia (possible bone‑marrow involvement). | WBC 12.5 × 10⁹/L, neutrophilia – supports SJS/TEN. |
| C‑reactive protein (CRP) & ESR | Gauge systemic inflammation; trend for response to therapy. | CRP 48 mg/L (elevated). |
| Comprehensive metabolic panel (electrolytes, renal, hepatic) | Baseline organ function; monitor for drug‑induced nephro‑/hepatotoxicity and fluid shifts. | Normal at baseline. |
| Serum albumin | Low albumin predicts poor prognosis (SCORTEN). | Not yet measured – should be ≤ 3 g/dL to increase SCORTEN score. |
| Urine output & renal ultrasound (if oliguria) | Early detection of acute kidney injury (AKI) from fluid loss or drug toxicity. | 1.8 mL/kg/h (adequate). |
| Blood cultures | Rule out secondary sepsis, especially if fever persists. | No growth. |
| Viral serology (HSV‑1/2, CMV, EBV, Mycoplasma pneumoniae) | Identify infectious triggers that may mimic or coexist. | Negative. |
| Skin biopsy (H&E, possibly direct immunofluorescence) | Confirm diagnosis, differentiate from EM, bullous pemphigoid, etc. | Shows full‑thickness necrosis. |
| SCORTEN calculation (within 24 h) | Predict mortality; includes age > 40 yr, malignancy, > 10 % BSA, serum urea > 10 mmol/L, glucose > 14 mmol/L, bicarbonate < 20 mmol/L, serum creatinine > 177 µmol/L. | Patient scores 1 (BSA > 10 %). |
| Ophthalmology assessment | Baseline ocular involvement; guide early interventions (lubricants, topical steroids). | Conjunctival injection, no corneal ulceration. |
| Gynecological exam (if female) / genital assessment | Document genital erosions; plan for local care. | Erosions present. |
These investigations establish baseline severity, identify organ dysfunction, and help risk‑stratify the patient (SCORTEN) for prognostication and therapeutic intensity.
4. Outline the evidence‑based acute management plan, including pharmacologic and supportive measures, citing the latest guideline.
Guideline reference: American Academy of Dermatology (AAD) Clinical Guidelines for the Management of Stevens‑Johnson Syndrome/Toxic Epidermal Necrolysis, 2023 (updated 2023) and British Association of Dermatologists (BAD) SJS/TEN Guidelines, 2022. Both recommend early drug withdrawal, supportive care in a specialized unit, and consider immunomodulatory therapy on a case‑by‑case basis.
A. Immediate steps
- Discontinue all non‑essential drugs – stop lamotrigine immediately; review medication list for other possible culprits (e.g., ibuprofen).
- Transfer to a high‑dependency or burn unit – provides temperature‑controlled environment, sterile wound care, and multidisciplinary team (dermatology, intensive care, ophthalmology, infectious disease).
B. Supportive care (cornerstone)
| Component | Details |
|---|---|
| Fluid and electrolyte management | Replace insensible losses similar to burn patients: calculate fluid needs as 2 mL/kg/%BSA (≈ 2 × 70 kg × 12 % = 1.68 L) over the first 24 h, then adjust based on urine output (target ≥ 0.5 mL/kg/h) and serum electrolytes. Use isotonic crystalloids (Ringer’s lactate). |
| Temperature regulation | Maintain ambient temperature 22‑24 °C; use warming blankets if hypothermic, cooling measures if hyperthermic. |
| Wound care | Non‑adhesive, sterile, semi‑occlusive dressings (e.g., silicone‑based). Debride only necrotic tissue that is clearly detached; avoid aggressive debridement. |
| Pain control | IV paracetamol 1 g q6h; if inadequate, add low‑dose morphine infusion (titrate to pain score). Avoid NSAIDs due to potential cross‑reactivity. |
| Nutritional support | Early enteral feeding (high‑protein, 1.5 g/kg/day) via nasogastric tube if oral intake < 50 % of needs. |
| Infection prophylaxis | No routine antibiotics; obtain cultures if fever > 38.5 °C persists > 48 h or if clinical signs of infection appear. |
| Ophthalmic care | Frequent lubricating eye drops (preservative‑free artificial tears q2h), topical steroids (prednisolone acetate 1 % q6h) if conjunctival inflammation, and referral for amniotic membrane transplantation if corneal involvement develops. |
| Genital care | Gentle cleansing, topical mupirocin ointment, and analgesic mouthwash (e.g., chlorhexidine 0.12 %). |
| Psychological support | Counsel patient and family; involve mental‑health services early. |
C. Pharmacologic immunomodulation (selected based on severity)
| Agent | Evidence & guideline stance (2023 AAD) | Dosing (adult) |
|---|---|---|
| Cyclosporine | Strongest evidence among immunosuppressants (randomized trials show reduced mortality, faster re‑epithelialization). Recommended as first‑line if started within 48 h of onset. | 3 mg/kg/day IV divided q12h (target trough 150‑200 ng/mL). |
| Intravenous immunoglobulin (IVIG) | Mixed data; high‑dose (> 2 g/kg) may be considered when cyclosporine contraindicated. 2023 AAD gives a conditional recommendation. | 2 g/kg total, given as 400 mg/kg/day over 5 days. |
| Systemic corticosteroids | Controversial; some centers use high‑dose methylprednisolone (1‑2 mg/kg/day) for < 7 days, especially if early presentation. 2022 BAD suggests use only in early disease (< 48 h) and when no contraindications. | Methylprednisolone 1 mg/kg IV q24h. |
| TNF‑α inhibitors (e.g., etanercept) | Emerging data (small RCTs) show faster skin healing; 2023 AAD lists as optional for refractory cases. | Etanercept 50 mg subcutaneously on day 1, repeat on day 7 if needed. |
In this patient (SJS/TEN overlap, SCORTEN = 1, early presentation < 48 h), the guideline‑preferred approach is IV cyclosporine combined with aggressive supportive care.
D. Monitoring
- Vital signs q4 h; temperature, HR, BP, SpO₂.
- Fluid balance – strict input/output chart; daily weight.
- Laboratory panel – CBC, electrolytes, renal & liver function daily for the first 5 days, then every 48 h.
- SCORTEN reassessment on day 3.
- Ophthalmology review daily until stable.
E. Discharge criteria
- Re‑epithelialization > 90 % of denuded areas.
- Stable vitals for ≥ 24 h,
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