What are the symptoms of Stevens-Johnson Syndrome (SJS)?

From the Guidelines

Stevens Johnson Syndrome (SJS) is a severe skin reaction characterized by flu-like symptoms initially, including fever, sore throat, cough, and burning eyes, which can progress to painful red or purplish rash, blisters, and skin shedding, affecting the skin and mucous membranes. The symptoms of SJS typically start with a prodrome of fever, malaise, and upper respiratory tract symptoms, which can be difficult to distinguish from a precipitating infection 1. Some key features of SJS include:

• Cutaneous pain as a prominent early feature, which should alert the physician to incipient epidermal necrolysis 1
• Involvement of the mucous membranes of the eyes, mouth, nose, and genitalia, leading to an erosive and haemorrhagic mucositis 1
• Lesional skin that is tender to touch, with minimal shearing forces causing the epidermis to peel back, demonstrating the Nikolsky sign 1
• Blistering and extensive necrolysis, resulting in the detachment of sheets of epidermis, leaving areas of exposed dermis 1
• Denuded dermis that exudes serum, becomes secondarily infected, and readily bleeds 1 It is essential to recognize these symptoms early, as SJS can lead to serious complications, including sepsis, organ failure, and death if not promptly treated 1. The mortality rate for SJS ranges from 5-15%, highlighting the importance of early recognition and intervention 1. Immediate hospitalization and referral to a specialist unit, such as a burn center, are crucial for managing SJS and reducing the risk of complications and mortality 1.

From the Research

Symptoms of Stevens Johnson Syndrome

The symptoms of Stevens Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) include:

• Mucous membrane and epidermal detachment 2
• Skin infections 3
• High morbidity and mortality rates 4
• Severe cutaneous, mucosal, and systemic manifestations 4
• Rapidly progressing systemic disease 4

Causes of Stevens Johnson Syndrome

The causes of SJS and TEN include:

• Drug-induced reactions, particularly:
  • Antimicrobials 2
  • Antiepileptics 2
  • Allopurinol 2
  • Nonsteroidal anti-inflammatory medications 2
  • Carbamazepine 5
• Immune dysregulation 2
• Active malignancy 2
• Genetic predisposition 2

Treatment of Stevens Johnson Syndrome

The treatment of SJS and TEN includes:

• Corticosteroids 5, 3, 6
• Intravenous immunoglobulin (IVIG) therapy 5, 3, 6
• Combination therapy of IVIG and corticosteroids 5, 3, 6
• Supportive care in an intensive care unit or burn unit 2