What is Steven Johnson syndrome and how is it treated?

From the Guidelines

Stevens-Johnson syndrome (SJS) is a rare, serious skin and mucous membrane disorder that requires immediate discontinuation of the suspected triggering medication and hospitalization, with supportive care as the cornerstone of treatment, including fluid replacement, nutritional support, wound care, and pain management, as emphasized by the British Association of Dermatologists' guidelines 1.

Key Aspects of Treatment

• Supportive care is the most important aspect in the treatment of patients of all ages with SJS/TEN, including care of skin, mucous membranes, resuscitation, fluid balance, nutritional support, analgesia, and preventing life-threatening complications and long-term morbidity 1.
• Treatment should be provided in a multidisciplinary team (MDT) setting, with specialists from dermatology, intensive care, ophthalmology, and specialist skincare nursing, among others 1.
• Children and young people with SJS/TEN should be managed in age-appropriate specialist units with an MDT, and may require transfer to specialized care, such as a pediatric burn center or a specialized dermatology service PICU 1.

Fluid Replacement and Wound Care

• Fluid replacement is crucial to prevent end-organ hypoperfusion and shock, with requirements lower than those predicted by the Parkland formula, and can be determined by the formula: body weight/% BSA epidermal detachment 1.
• Wound care should include sterile dressings, and topical treatments such as corticosteroids, calcineurin inhibitors, and antibiotics may be considered, although their effectiveness is still being studied 1.

Immunomodulatory Treatments

• The use of immunosuppressive treatments, such as corticosteroids, cyclosporine, or intravenous immunoglobulin (IVIG), remains somewhat controversial, with some studies suggesting potential benefits, but also risks, such as increased risk of sepsis 1.

Eye Care

• Eye care is crucial, with ophthalmology consultation and topical lubricants or antibiotics often required to prevent permanent damage, and should be prioritized to reduce the risk of vision impairment 1.

Outcome and Prognosis

• The condition is life-threatening, with mortality rates of 5-15%, making rapid diagnosis and treatment essential to improve outcomes and reduce complications like sepsis, permanent skin damage, and vision impairment 1.

From the Research

Definition and Overview of Steven Johnson Syndrome

• Steven Johnson syndrome (SJS) is a rare, yet life-threatening, delayed-type hypersensitivity reaction characterized by mucocutaneous epidermal necrolysis 2.
• It is associated with the use of certain medications, such as sulfonamides, antiepileptics, and some classes of nonsteroidal anti-inflammatory drugs 2.
• Toxic epidermal necrolysis (TEN) is a severe manifestation of SJS, defined as greater than 30% skin detachment 2.

Treatment of Steven Johnson Syndrome

• The use of corticosteroids in the treatment of SJS remains controversial, but studies have shown that they can be beneficial in treatment of the syndrome 3.
• Intravenous immunoglobulin (IVIG) treatment is commonly used to treat SJS and TEN, with some studies suggesting that it can reduce recovery time and mortality 4, 5, 6.
• A meta-analysis of 26 studies found that the combination of IVIG and corticosteroid markedly reduced the recovery time and hospitalization length compared with solo corticosteroid group 4.
• Another study found that IVIG combined with corticosteroids tended to reduce the time to arrest of progression and the length of hospital stay, and the mortality rate was 45% lower for patients treated with IVIG combined with corticosteroids than those only treated with corticosteroid therapy 5.

Management and Prognosis

• Prompt identification and diagnosis of SJS are crucial, and emergency providers should have a comprehensive knowledge of adverse cutaneous drug reactions 2.
• The severity of SJS and TEN can be determined using the SCORTEN system, which can help predict mortality rates 6.
• High-dose IVIG (≥2 g/kg) appears to significantly decrease mortality in patients with SJS or TEN 6.
• The incidence of skin infections was significantly lower in the combined therapy group, and the total infection rate of patients treated with combination therapy tended to decrease compared to patients treated with corticosteroids alone 5.