Disease Description (Causes and Symptoms)
- Definition: Stevens–Johnson syndrome (SJS) is a rare but potentially life-threatening immune-mediated systemic mucocutaneous disease characterized by acute necrotic detachment of the skin and mucous membranes (epidermal detachment). More extensive cases are classified as toxic epidermal necrolysis (TEN).
- Causes: It is most commonly caused by medications (anticonvulsants, antibiotics—especially sulfonamides, some penicillins, and some antigout medications), and more rarely by infections (e.g., Mycoplasma pneumoniae and viruses) or with no identifiable cause (idiopathic).
- Pathophysiology: Medications or infections activate specific T cells, causing apoptosis (cell death) of keratinocytes, separation at the dermal-epidermal junction, and extensive epidermal necrosis.
- Main symptoms:
- After initial systemic symptoms (fever, malaise), erythematous macules and targetoid lesions, blister formation, and extensive epidermal detachment (ranging from part of the body surface to extensive involvement)
- Involvement of multiple mucous membranes, including the oral cavity, conjunctiva, and genitals (painful ulcers and hemorrhagic crusts)
- Ocular complications (conjunctivitis, symblepharon, keratoconjunctivitis sicca, etc.)
- In severe cases, dyspnea, nutritional deficiencies due to malabsorption, secondary infection, and sepsis may occur
Treatment
- Immediate measures
- Immediately discontinue the suspected causative medication (when possible, check prescription records and when the medication was started)
- If symptoms are severe or there is extensive epidermal detachment, transfer the patient to a specialized burn center or intensive care unit
- Supportive care (the core of treatment)
- Hospital bed care, pain management, fluid, electrolyte, and nutritional supplementation (high-protein, high-calorie), and monitoring of body temperature and electrolytes
- Skin care: Aseptic wound care (such as hydrocolloid or non-adherent dressings), removal of necrotic tissue, and prevention of secondary infection
- Infection management: Appropriate antibiotics should be used selectively when infection is present (prophylactic broad-spectrum antibiotics are not recommended)
- Ophthalmic treatment: Artificial tears and eye drops (antibiotic and anti-inflammatory); when necessary, prevention of adhesions and specialized surgery and reconstruction
- Systemic drug treatment (evidence and considerations for use)
- Systemic corticosteroids: Their use in the early stage remains controversial. Short-term use in the early acute phase followed by rapid tapering may be considered, but careful judgment is needed because of the possibility of increased infection risk.
- Intravenous immunoglobulin (IVIG): Used in some cases, but evidence of its effectiveness is mixed. Consideration depends on severity and timing.
- Cyclosporine: Recent studies have reported that early administration may inhibit progression of epidermal detachment and improve mortality rates (to be considered according to the center and the patient’s condition).
- Plasmapheresis and biologic agents (such as TNF-α inhibitors): Reported in some cases; evidence is limited, and these treatments should be performed after multidisciplinary discussion for severely ill patients.
- Precautions and self-care
- Be sure to record and avoid the causative medication (a medication allergy card or bracelet is recommended)
- Avoid ultraviolet exposure and physical irritation while the skin and mucous membranes recover
- If there are eye symptoms, receive immediate ophthalmic follow-up (important for preventing permanent vision loss)
- Complication management
- Specialized evaluation and treatment for respiratory, nutritional, and urinary tract problems
- Long-term: Changes in skin pigmentation, scars, dry eye and reduced vision, and psychosocial sequelae
Checkpoints Before an Outpatient Visit (Including Example Questions)
- Items to prepare (recommended to bring)
- A list of all medications currently being taken (including prescription drugs, over-the-counter medications, herbal medicines, and health functional foods), and the dates they were started and stopped, if possible
- Records of past allergies and drug reactions
- Photos from the time symptoms began (skin and mucosal changes), and a symptom diary (fever, pain, difficulty breathing, etc.)
- Recent test results (blood tests, kidney function, etc.) or records of hospitalization
- Self-check before an outpatient visit (when to go to the emergency room immediately)
- If there is difficulty breathing, rapid heartbeat or low blood pressure, extensive epidermal detachment (a large body surface area), rapidly worsening vision, high fever, or changes in consciousness, go to the emergency room immediately
- Example questions (items to mention first when starting the consultation)
- “I have started taking several medications recently, and I will give you the list.”
- “This is when my symptoms started and how quickly they progressed (including photos).”
Examples of General Questions to Ask the Doctor
- Can you determine exactly which medication caused these symptoms? Which medication is suspected?
- How do you assess my current condition (severity)? Do I need hospitalization or transfer to a specialized center?
- What are the advantages, disadvantages, and expected effects of the treatment options (conservative supportive care, corticosteroids, IVIG, cyclosporine, etc.)?
- What should I do to prevent complications (especially ocular complications and secondary infection)?
- When can I return to daily life, and how should I manage the risk of recurrence?
Examples of Disease-Specific Questions
- What additional tests (e.g., drug provocation testing, or investigation of past medical history or medication exposure) do you recommend to identify the suspected triggering medication?
- I had ocular involvement. What ophthalmic follow-up and treatment (e.g., regular use of lubricants and the need for surgical treatment by an ophthalmologist) are recommended to protect my long-term vision?