What is Stevens-Johnson Syndrome (SJS)?

From the Guidelines

Stevens-Johnson Syndrome (SJS) is a rare, severe mucocutaneous reaction characterized by blistering and epithelial sloughing, usually triggered by drugs or infections.

Key Features

• SJS is part of a severity spectrum that includes Toxic Epidermal Necrolysis (TEN), with SJS being the less extensive form and TEN being the more extensive form 1
• The condition is marked by epidermal loss and multisite mucositis, accompanied by systemic symptoms such as fever, malaise, and upper respiratory tract symptoms 1
• Mucosal involvement is a key feature, with the mucous membranes of the eyes, mouth, nose, and genitalia often being affected early in the course of the disease 1
• Cutaneous pain is a prominent early symptom, and the presence of this symptom should alert the physician to the possibility of incipient epidermal necrolysis 1

Clinical Phenotypes

• SJS is characterized by epidermal detachment of less than 10% of the body surface area (BSA), plus widespread purple/red macules or flat atypical targets 1
• Overlap SJS-TEN is characterized by detachment or skin necrosis of 10-30% BSA, plus widespread purpuric macules or flat atypical targets 1
• TEN is characterized by detachment or skin necrosis of greater than 30% BSA 1

Diagnosis and Management

• Diagnosis is often based on clinical presentation, but skin biopsy or frozen section of a blister roof may be necessary to confirm the diagnosis and rule out other conditions such as staphylococcal scalded skin syndrome (SSSS) or immunobullous disorders 1
• Management of SJS/TEN typically involves withdrawal of the offending drug, supportive care, and treatment of any underlying infections or other complications 1

From the Research

Definition and Characteristics of Stevens-Johnson Syndrome (SJS)

• Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) are severe mucocutaneous reactions that are primarily drug-induced, characterized by significant morbidity and mortality 2, 3, 4, 5, 6.
• These conditions manifest in extensive skin detachment, distinguishing them from other generalized skin eruptions 2.
• SJS and TEN are considered variants of one disease entity with varying degrees of severity called epidermal necrolysis (EN) 3.

Causes and Triggers of SJS

• The majority of SJS/TEN cases are associated with medication or infection 5.
• Medications are the most common trigger for SJS/TEN, with certain drugs being more commonly implicated than others 2, 4.

Clinical Presentation and Diagnosis of SJS

• SJS/TEN is characterized by widespread skin and mucosal necrosis and detachment, which can be triggered by medications 4.
• Evaluation of the skin, mucosal, pulmonary, renal, genital, and ocular systems are essential in the diagnosis of SJS/TEN, as well as in the identification of complications (e.g., sepsis) 5.
• Laboratory and radiological testing cannot confirm the diagnosis in the emergency department setting, but they may assist in the identification of complications 5.

Management and Treatment of SJS

• Early recognition and immediate withdrawal of causative agents, and critical multidisciplinary supportive care are key management of SJS/TEN 4.
• Systemic corticosteroids remain one of the most common treatment options for SJS/TEN, though the efficacy remains uncertain 4.
• Cyclosporine and TNF-α inhibitors have been shown to decrease the mortality of SJS/TEN, and a combination of intravenous immunoglobulin (IVIg) and corticosteroids is most effective for SJS/TEN overlap and TEN 4, 6.