From the Guidelines
Stevens-Johnson Syndrome (SJS) is a severe skin reaction characterized by flu-like symptoms initially, including fever, sore throat, cough, and fatigue, which can progress to painful red or purple rash, blisters, and skin peeling, affecting the skin and mucous membranes, as described in the British Association of Dermatologists' guidelines 1. The symptoms of SJS can be divided into several key areas, including:
- Flu-like symptoms such as fever, sore throat, cough, and fatigue, which can precede the skin manifestations by several days 1
- Painful red or purple rash that spreads and blisters, affecting the skin and mucous membranes, particularly on the mouth, lips, eyes, and genitals 1
- Skin peeling and sloughing in large sheets, resembling severe burns, which can lead to exposed dermis and increased risk of infection 1
- Mucosal involvement, including erosive and hemorrhagic mucositis, which can affect the eyes, mouth, nose, and genitalia 1
- Systemic symptoms, such as malaise, upper respiratory tract symptoms, and cutaneous pain, which can indicate the severity of the condition 1 The condition is a medical emergency requiring immediate hospitalization, as it can lead to serious complications including sepsis, organ failure, and death if not promptly treated, with the severity determined by the percentage of body surface area affected 1. Some key points to note include:
- The importance of taking a detailed history from patients with SJS/TEN, including symptoms suggestive of the condition, previous medical problems, and potential triggers such as medications or infections 1
- The need to exclude differential diagnoses, such as staphylococcal scalded skin syndrome (SSSS), through clinical assessment and skin biopsy if necessary 1
- The potential for long-term sequelae, particularly ophthalmic, mucocutaneous, and psychological, in survivors of SJS/TEN, which can be severely debilitating 1
From the Research
Symptoms of Stevens-Johnson Syndrome
- Mucous membrane and epidermal detachment 2, 3
- Widespread full-thickness cutaneous and mucosal necrosis 4
- Skin loss similar to second-degree burns 5
- Mucocutaneous vesiculobullous disorders 4
- Severe eye problems, including ocular scaring 2, 4
Causes and Risk Factors
- Medications, particularly antimicrobials, antiepileptics, allopurinol, and nonsteroidal anti-inflammatory medications 2, 3
- Infections 2
- Genetic predisposition 3
- Immune dysregulation 3
- Active malignancy 3
Treatment and Management
- Supportive management, including fluid resuscitation, nutritional supplementation, and meticulous wound care 2, 5
- Discontinuation of the offending drug 2, 5
- Systemic therapies, such as glucocorticoids, intravenous immunoglobulins (IVIGs), cyclosporin, and tumour necrosis factor-alpha (TNF-alpha) inhibitors 3, 6
- Multidisciplinary team care, including dermatology, ear, nose, and throat surgery, ophthalmology, urology, colorectal surgery, and gynecology 4