What is Stevens-Johnson syndrome (Toxic Epidermal Necrolysis)?

From the Guidelines

Stevens Johnson Syndrome (SJS) is a life-threatening condition that requires immediate hospitalization and supportive care, including fluid replacement, wound care, and sometimes specialized burn unit care, as stated in the UK guidelines for the management of SJS/TEN in adults 2016 1.

Key Considerations

• SJS is a rare, serious skin and mucous membrane disorder typically triggered by medications or infections.
• It's characterized by a painful red or purplish rash that spreads and blisters, eventually causing the top layer of skin to die and shed.
• Common medication triggers include certain antibiotics (like sulfonamides), anticonvulsants, and some pain relievers.
• If suspected, seek emergency medical attention immediately.

Treatment and Management

• Treatment involves stopping the causative medication and providing supportive care.
• Supportive care includes fluid replacement, wound care, and sometimes specialized burn unit care, as recommended by the UK guidelines 1.
• The use of an appropriate dressing on exposed dermis can reduce fluid and protein loss, limit microbial colonization, and help with pain control, as suggested in the guidelines 1.
• A surgical approach involving debridement of detached epidermis and physiological wound closure using biosynthetic xenograft or allograft may be considered in some cases, as mentioned in the guidelines 1.

Complications and Prevention

• Complications can affect the eyes, lungs, and other organs.
• The mortality rate ranges from 5-15%, with most deaths resulting from infections or respiratory failure, as noted in the guidelines 1.
• Recovery can take weeks to months, and some patients may experience long-term complications like vision problems or skin discoloration.
• Prevention involves avoiding medications that previously caused reactions and informing all healthcare providers about your history of SJS.

Ocular Involvement

• Local ocular measures are supportive and empirical, and ocular surface lubrication and conjunctival hygiene must be maintained throughout the acute illness, as recommended in the guidelines 1.
• Topical corticosteroids are commonly used to ameliorate conjunctival inflammation, and a grading system for acute ocular severity of SJS/TEN can be used to assess the severity of ocular involvement, as described in the guidelines 1.

Analgesia and Fluid Replacement

• Patient comfort should be ensured using the principles of the World Health Organization’s analgesic ladder, as suggested in the guidelines 1.
• Fluid resuscitation is crucial to prevent end-organ hypoperfusion and shock, and the predicted volume of fluid replacement can be determined by the formula: body weight/% BSA epidermal detachment, as recommended in the guidelines 1.

From the FDA Drug Label

Severe erythema multiforme (Stevens-Johnson syndrome)

• Stevens-Johnson syndrome is listed as one of the dermatologic diseases for which prednisone is indicated.
• The drug is used for the treatment of Severe erythema multiforme (Stevens-Johnson syndrome) 2.

From the Research

Definition and Characteristics of Stevens Johnson Syndrome

• Stevens-Johnson syndrome (SJS) is a serious condition characterized by necrosis of the skin and mucus membranes, mainly caused by medication and infections 3.
• SJS is considered a delayed-type hypersensitivity reaction to drugs, representing a true medical emergency that requires early recognition and appropriate management 4.
• The difference between SJS, SJS/TEN overlap, and TEN is defined by the degree of skin detachment: SJS is defined as skin involvement of < 10%, TEN is defined as skin involvement of > 30%, and SJS/TEN overlap as 10-30% skin involvement 4.

Causes and Risk Factors

• Drugs with a high risk of causing SJS/TEN include anti-infective sulfonamides, anti-epileptic drugs, non-steroidal anti-inflammatory drugs of the oxicam type, allopurinol, nevirapine, and chlormezanone 4.
• Besides conventional drugs, herbal remedies and new biologicals should be considered as causative agents 4.
• The increased risk of hypersensitivity reactions to certain drugs may be linked to specific HLA antigens 4.

Diagnosis and Treatment

• The diagnosis of different degrees of epidermal necrolysis is based on the clinical assessment in conjunction with the corresponding histopathology 4.
• There is no established therapy for SJS/TEN, but corticosteroids and intravenous immunoglobulin (IVIG) have been utilized as immunomodulators 3.
• Early treatment with high-dose steroids, including methylprednisolone pulse therapy, and IVIG together with corticosteroids are possible therapeutic options to improve the prognosis of SJS/TEN 3.
• Systemic immunomodulating therapies, including glucocorticosteroids, intravenous immunoglobulins, cyclosporine, and others, have been used to treat SJS/TEN, with glucocorticosteroids and cyclosporine showing promising results 5.

Complications and Management

• The most common complications of SJS/TEN are ocular, cutaneous, or renal, with nasopharyngeal, esophageal, and genital mucosal involvement also playing a role 4.
• Septicemia is a leading cause of morbidity and fatality in the acute phase of SJS/TEN 4.
• The acute management of SJS/TEN requires a multidisciplinary approach, with immediate withdrawal of potentially causative drugs and prompt referral to an appropriate medical center for specific supportive treatment being mandatory 4.