Última actualización:
4/19/2024
Años publicados: 1986, 1989, 1996, 1998, 1999, 2004, 2007, 2015, 2018, 2024
NORD gratefully acknowledges Hemali Shah, MD, Medical University of South Carolina, Sofia Labbouz, BMBS, Sheffield Teaching Hospitals NHS Foundation Trust, Rose Parisi, Albany Medical College, Roni P. Dodiuk-Gad, MD, Clinical Associate Professor & Head, Dermatology Program, Bruce Rappaport Faculty of Medicine, Technion – Institute of Technology, Israel; Head, Inflammatory and Autoimmune Skin Diseases Unit, Dermatology Department, Emek Medical Center, Israel, and Dr. med Maja Mockenhaupt, Dokumentationszentrum schwerer Hautreaktionen, Department of Dermatology, University of Freiburg Medical Center, for assistance in the preparation of this report.
Summary
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare severe cutaneous (skin) adverse drug reactions. They represent a spectrum of disease and are differentiated primarily based on body surface area (BSA) involvement, although they can both be life-threatening. The consensus definition published in 1993 states that SJS affects less than 10% of the body surface area, while TEN affects more than 30% of the body surface area. The term SJS/TEN-overlap syndrome is used to describe cases in which 10%-30% of the body surface area is involved. The reaction may start with a fever and non-specific flu-like symptoms followed by the appearance of erythematous macules (red spots) that may cover a large part of the body, and painful blistering of the skin and mucous membranes with possible involvement of the eyes, lips, mouth, nose and genitalia. Internal organ involvement may occur and lead to multi-organ failure.
Numerous drugs have been reported to cause SJS and TEN, namely allopurinol, anticonvulsants (anti-epileptics), sulfonamide medications, antibiotics such as minocycline, non-steroidal anti-inflammatory drugs, anti-HIV and anti-tuberculosis (TB) medications. However, approximately 25% of cases are not drug-induced but are caused by infections or deemed idiopathic (of unknown cause).
Individuals with suspected SJS or TEN should immediately stop taking the suspected offending drug and all non-essential medications. Prompt recognition and early hospitalization to provide supportive and pharmacological treatment are essential. It is also important to note that survivors of SJS/TEN suffer from long-term emotional and physical complications.
Introduction
SJS and TEN are classified as severe cutaneous adverse reactions (SCAR), a subcategory of adverse drug reactions that can be life-threatening and are immunologically mediated. The disorders discussed in this report are generally broken down into SJS, SJS/TEN overlap and TEN. SJS was first described in the medical literature in 1922 by doctors A.M. Stevens and F.C. Johnson. The term toxic epidermal necrolysis was introduced in the medical literature in 1956 by Dr. A. Lyell and is also known as Lyell syndrome.
Most cases of SJS/TEN begin with the development of general, non-specific symptoms including flu-like symptoms, fever, burning or stinging of the eyes, body aches and discomfort or difficulty swallowing. Additional non-specific symptoms include headaches, chills, joint pain and a general feeling of poor health (malaise). A pus-producing (purulent) cough that also brings up mucous, phlegm and saliva (sputum) may also occur. These symptoms usually precede the development of skin involvement by a few days and characteristically happen one to eight weeks after exposure to the trigger.
The initial skin manifestation is often the development of a superficial reddening of the skin (erythema) or reddish spots on the skin (macules) which can look purple (violaceous) in darker skin tones. These lesions often rapidly spread (coalesce). In some people, these lesions may resemble a bull’s eye, so-called “target” lesions. The rash may be itchy (pruritic) but is usually painful. Blisters may appear leading to detachment and sloughing of the skin, leaving open wounds (erosions).
Skin involvement is often accompanied by inflammation of the external mucous membranes such as the mouth, lips, eyes and genitalia. Blisters may also form on internal mucous membranes of the body including the lining inside of the mouth (stomatitis), nose, airway, esophagus, urinary tract and internal genitalia. Mucous membrane involvement can precede or follow skin symptoms and often begins with soreness, then little blisters that rupture and leave erosions that may bleed and be painful. Involvement of the lips and inside of the mouth can result in painful mucosal lesions and extensive ulceration which are usually associated with crusted, bleeding lips and this can make eating difficult. Lesions in the genitourinary tract can cause diminished urine flow or an inability to pass urine due to pain. Airway involvement can cause discomfort or difficulty with breathing and may predispose to upper respiratory tract infections. Involvement of the eyes can cause pain, redness, weeping and crusting of the eyelids. Affected individuals may also experience pain and reddish discoloration in the whites of the eyes (sclera). Conjunctivitis, which is inflammation of the thin, clear membrane that lines the inner surface of the eyelids and the outer surface of the eye, is common. Swelling due to fluid accumulation (eyelid edema) may also occur. Eye crusts may form and there may be a sensation of sand or grit in the eyes. Affected individuals may be abnormally sensitive to light (photophobia). Skin and mucous membrane involvement initially can be mild, or it can rapidly progress in severity. Eventually, the upper layer of the skin (epidermis) may pull away (detach) from the lower layer (dermis), leaving erosions. In SJS, this affects less than 10% of the body surface area while in TEN there is more widespread skin involvement with more than 30% of body surface area affected by skin loss. SJS/TEN-overlap is characterized by 10-30% of body surface area involvement. Scarring and secondary infection may occur in the affected areas if not treated appropriately. Affected individuals can potentially develop sepsis, a widespread, life-threatening condition that results from infection spreading in the blood and/or other body tissues, leading to a widespread inflammatory reaction in the body and potential systemic organ dysfunction. Individuals with TEN may resemble patients with severe burns in physical appearance, metabolic abnormalities and high critical care needs. The mortality rate of SJS/TEN is 10-30%.
Late Complications
Most individuals who survive SJS or TEN will suffer from long-term emotional and physical complications. Long-term skin issues can include itching (pruritus), excessive sweating (hyperhidrosis) and abnormal dryness of the skin. Abnormal lightening or darkening of affected areas of skin (hypopigmentation and hyperpigmentation, respectively) and scarring can occur secondary to skin inflammation. Permanent loss of nail beds may occur, or the fingernails and toenails may grow back abnormally (onychodystrophy). Chronic eye abnormalities can include chronic inflammation, abnormal dryness of the cornea (corneal xerosis), dry eye syndrome, folding inward of one or both eyelids (entropion), inverted eyelashes (trichiasis) and one or both eyelids may become stuck to the eye surface (symblepharon). Eye tissue can be significantly damaged resulting in scarring, vision loss and in rare cases, blindness. Involvement of the mucous membranes lining the respiratory and digestive tracts can lead to lung damage, bronchitis, chronic obstructive pulmonary disease and scarring of the esophagus. Dental complications including dry mouth (xerostomia), inflammation of the gums and gum disease have been observed due to changes in the quantity and quality of saliva. Genitourinary abnormalities can include urethral erosions and inflammation of the head of the penis (balanitis) in males and narrowing of the opening of the vagina and abnormal sticking together (adhesion) of the vaginal lips (labia) so that the vagina appears narrow or closed (vaginal synechia) in females. Urethral erosions and scarring or narrowing may also occur in females.
Emotional long-term complications are common in SJS/TEN survivors. Survivors are reported to experience a high rate of post-traumatic stress disorder (PTSD), anxiety and depression. One study estimated that over 50% of survivors screen positive for depression, over 40% of survivors screen positive for anxiety and about 20% of survivors screen positive for PTSD. Emotional complications are important to assess and treat appropriately.
SJS and TEN are life-threatening reactions that are predominantly drug-induced. Approximately 75% of SJS and TEN cases are caused by medications, but this percentage varies according to age, with a higher percentage in adults and a lower percentage in children. The drugs most associated with these disorders include allopurinol, a drug commonly used to treat gout or kidney stones; anti-convulsants (anti-epileptics) including phenytoin, carbamazepine, lamotrigine and phenobarbital; sulfonamide medications; antibiotics such as minocycline; nonsteroidal anti-inflammatory drugs (NSAIDs); anti-HIV medications such as nevirapine and anti-tuberculosis (TB) medications. In the last few years, multiple new anticancer treatments were developed and have also been recognized as an important trigger for adverse drug reactions. Various other drugs have been reported as triggers of SJS and TEN, and almost any medication can cause SJS/TEN, including over-the-counter medications and herbal/natural preparations.
In addition to medication being the dominant trigger of SJS and TEN, other predisposing factors are necessary to induce this type of rare reaction. There is a genetic predisposition to develop this disorder in certain ethnic groups. A genetic predisposition means an individual has specific gene variants that increases the risk of developing a specific type of reaction. It is well known that certain human leukocyte antigens (HLA), proteins found on most cells in the body which allow the immune system to recognize which cells belong in the body and which are foreign, are predisposing factors for developing SJS/TEN when taking certain medications. For example, HLA B*1502 conveys a risk of developing SJS/TEN in persons of Chinese or Southeast Asian ancestry when taking the drug carbamazepine, an anti-seizure medication. Genetic screening prior to prescription of high-risk drugs is an important preventive measure well incorporated in many Asian countries and was proven to decrease the incidence of SJS/TEN and other SCARs. Unfortunately, these genetic screening tests are not yet incorporated in routine clinical practice in other parts of the world including North America and Europe. In recent years, additional HLA associations have been identified, please see Table 1.
Table 1. Human Leukocyte Antigen (HLA) Association Between Drugs and SJS/TEN in Different Ethnicities
(adapted from Chang et al. 2020 and Pavlos et al. 2017)
| Generic drug (brand name) | HLA allele | Ethnicity |
| Allopurinol (Zyloprim, Lopurin, Zyloric, Uricto) | B*58:01 |
Han Chinese, Thai, African American, European, Israeli |
| Carbamazepine (Tegretol, Curatil, Carbatrol, Epitol, Equetro) | B*15:02
B*15:11 |
Han Chinese, Thai, Indian, Malaysian, Vietnam, Singapore, Hong Kong Koreans, Japanese European |
| Oxcarbazepine (Trileptal, Oxtellar) | B*15:02 and B*15:18 | Han Chinese, Taiwanese |
| Lamotrigine (Lamictal) | B*15:02 B*38:01 |
Han Chinese Spanish |
| Phenytoin (Dilantin, Phenytek, Epanutin) | B*15:02, Cw*08:01 and DRB1 *16:02 B*13:01, B*56:02/04, CYP2C19*3 CYP2C9*3 B*51:01 |
Han Chinese Thai Spanish Malaysians |
| Nevirapine (Viramune) | C*04:01 | Malawian |
| Co-trimoxazole (Septra, Bactrim, Sulfatrim, Cotrim) | HLA-B*15:02, HLA-C* 06:02, and HLA-C* 08:01 | Thailand |
| Strontium ranelate (Aristo, Protelos) | HLA-A*33:03, HLA-B* 58:01 | Han Chinese |
| Sulfamethoxazole (Gantanol) | B*38 | European |
SJS and TEN can affect individuals of any age, but the incidence is much higher in the elderly population. This may be related to increased exposures to potentially causative medications. Individuals of every race and ethnicity can develop these disorders; however, it is more common in Asian populations. The incidence in western societies is 1-2 per 1,000,000 people in the general population.
There are no specific blood tests yet to diagnose SJS/TEN. The diagnosis is based upon identification of characteristic signs and symptoms, a detailed history from the patient and/or relatives, a thorough physical examination and the results of blood tests and a skin biopsy. A biopsy involves taking a small sample of affected skin and examining it under a microscope. An additional skin biopsy for immunofluorescence (special staining) may also be performed to exclude other conditions such as autoimmune blistering disorders which may have similar manifestations to SJS/TEN, especially early on (please see list above).
Biological markers found in the blood and/or bulla fluid such as granulysin may in the future help early identification of SJS/TEN but they lack validation and are at present used as a research tool. Other in vitro and in vivo diagnostic tests are used in research settings.
A disease severity scoring system called SCORTEN (Score of TEN) has been established to help physicians assess the severity of illness. Other scoring systems such as ABCD-10 (age, bicarbonate, cancer, dialysis, 10% body surface area) and CRISTEN (clinical risk score for TEN) have also been more recently developed to predict the risk of mortality.
Treatment
The mainstay of management is early diagnosis, immediate cessation of the suspected culprit drug(s) and all other non-essential drugs, hospitalization and supportive management by doctors with experience in the management of severe cutaneous adverse reactions (SCARs). Treatment involves the coordinated efforts and expertise of multiple specialists in skin such as dermatologists and burn surgeons, and including intensive care doctors, ophthalmologists, urologists, gynecologists, immunologists, pediatricians, psychiatrists, nurses and other healthcare professionals.
There is a lack of high-level evidence to support specific therapeutic interventions. Due to the immunological basis of SJS/TEN, immunomodulating therapies have been thought to have a role but because they are rare diseases, it is difficult to conduct trials to assess the efficacy of treatment and most information is from retrospective studies. A variety of treatment approaches have been used including cyclosporine; systemic corticosteroids; intravenous immunoglobulins; and tumor necrosis factor antagonists (anti-TNF). There is a consensus that good supportive care is the cornerstone of management in the acute phase in both children and adults with SJS/TEN (see Table 2).
Table 2. Supportive Care in the Management of SJS/TEN Based on an International Multidisciplinary Consensus
(adapted from Bruggen et al. 2021)
| Location of care | A center with experience in SCARs e.g. dermatology department, burn unit, ICU with multi-disciplinary management according to patient’s needs. |
| Drug management | Immediate cessation of suspected culprit drug(s) and any other non-essential medications.
There are no standard guidelines for systemic therapies, but a variety of treatment approaches have been used including cyclosporine; systemic corticosteroids such as dexamethasone or methylprednisolone, especially in a pulsed fashion; intravenous immunoglobulins, especially in children in early stages of diseases; and tumor necrosis factor antagonists (anti-TNF) such as etanercept and infliximab. |
| Room settings | Ambient temperature controlled for humidity. |
| Pain | Assessment may necessitate a visual scale, analgesia. |
| Mental health | Psychiatric and/or psychological assessment if appropriate. |
| Hydration and nutrition | May require peripheral venous access and/or insertion of nasogastric tube for fluid resuscitation and nutritional support. |
| Prevention of thromboembolism | Pharmacological and non-pharmacological measures to diminish the risk of deep vein thrombosis and pulmonary embolism. |
| Prevention of infections | Hand hygiene and use of personal protective equipment. Skin swabs, dipstick urinalysis, blood culture to screen for infections as needed. Antibiotics only if there is evidence of infection and as per local microbiology protocols. Prophylactic (protective) use of antibiotics/antimicrobials is not recommended. |
| Skin care | Cover entire skin and eroded areas with non-adherent dressings and/or apply white petrolatum. Antiseptic solutions for wound cleaning. Blisters may be punctured for symptomatic relief but the blister roof should be kept in place and may act as a protective biological dressing. |
| Eye care | Regular lubricant eye drops without preservatives. May need surgical separation of adhesions (symblepharons), topical steroids and antibiotics or other treatments as per ophthalmology advice. |
| Care of genitalia | Paraffin-based ointments and daily foreskin mobilization in men. May require insertion of a urinary catheter if there is urinary retention or pain on passing urine. |
| Mouth care | Antimicrobial and analgesic mouthwashes. Paraffin-based ointments on the lips. |
Long-term follow up is recommended for survivors of SJS/TEN as proposed by an international multidisciplinary DELPHI-based consensus, please see Table 3. There is no set follow-up schedule as this should be individualized to suit each patient’s needs.
Table 3. Proposed Multidisciplinary Follow-Up
(adapted from Ingen-Housz-Oro et al. 2023)
| Timing | Specialist (as needed in each individual case) | Intervention |
| 2 months after acute phase | Dermatologist | Coordination of care, assessment and treatment of skin complications such as scars, advice about sun protection and emollients |
| | Ophthalmologist | Care of eye sequelae such as dry eyes (xerophthalmia) and scarring |
| | Psychiatrist/psychologist | Assessment of psychological well-being including sleep and mood status, screening for psychiatric complications such as post-traumatic stress disorder |
| | ENT (Ear, Nose and Throat) | Assessment and treatment of oropharyngolaryngeal complications such as adhesions (synechiae) and care of patients who required orotracheal intubation |
| | Pneumologist | Care of patients who required intubation in the acute phase and assessment for complications such as fibrotic lung disease. |
| | Gynecologist/Urologist | Care of patients with severe genital involvement or at risk of vaginal or urethral strictures |
| | Dietician | Support of nutritional requirements (frequent weight loss during acute phase) |
| | Social worker | May facilitate rehabilitation in the community and assessment of disability or need for financial support |
| 6 months after acute phase | Same specialties as above | As above, according to individual need |
| | Stomatologist/dentist | Treatment of chronic oral mucosal involvement, dry mouth (xerostomia), general dental health |
| | Allergy work-up | Patch tests, allergy card prohibiting use of all suspect drugs, lymphocytic transformation test and ELISpot test may be helpful if available |
| | Neurologist or pain specialist | In cases of chronic pain |
| 12 months after acute phase | Same specialties as above | As above, according to individual need |
| | Dermatology | Prolonged follow-up to screen patient needs and coordinate multidisciplinary care |
Prevention
Primary Prevention
It is advisable to discuss the use of any new over-the-counter medications and herbal/natural remedies with a doctor as the ingredients in many of these are not recorded or formally regulated. It is important to avoid the drug(s) that triggered SJS/TEN together with any potential cross-reacting medications. In some Asian countries, screening for some known human leucocyte antigen (HLA) risk variants is performed before starting medications that could potentially cause severe cutaneous adverse reactions (SCARs) in an attempt to prevent these reactions (see Table 1). Genetic screening is not yet in widespread clinical use in other parts of the world, including in North America and Europe.
Secondary Prevention
Early recognition of SJS/TEN is important to start treatment promptly and avoid long-term effects, but these severe drug reactions can be difficult to diagnose as they can mimic other disorders (see list above in “Disorders with Similar Symptoms”) in the early stages and there may be a long lag period between the start of a new medication and the onset of symptoms.
Tertiary prevention
Prevention of long-term effects is crucial and can be achieved with long-term multi-disciplinary follow-up as proposed in Table 3.
Due to the rarity and unpredictable nature of SJS and TEN, there is a lack of trials and high-level evidence to support specific therapeutic interventions. There have been only a few randomized controlled studies assessing the efficacy of adjuvant (complementary) immunomodulatory treatments for SJS/TEN: one compared the use of placebo to thalidomide and was terminated early due to excess deaths in the thalidomide group (Wolkenstein et al, 1998). The second study was a non-blinded trial investigating the use of etanercept (a TNF-alpha inhibitor) versus corticosteroids, which showed improved skin healing time in the etanercept group (Wang et al. 2017).
Other immunomodulating therapies have been used in uncontrolled studies (studies without a control group to compare the effects of the investigational treatment), including systemic corticosteroids (Kardaun et al. 2007; Schneck et al. 2008; Hirahara et al. 2013), intravenous immunoglobulin (IVIg) (Firoz et al. 2012; Lee et al. 2013), and ciclosporin (Valeyrie-Allanore et al. 2010; Singh et al. 2013; Kirchhof et al. 2014). Other treatments have been described in the literature but they have a limited strength of evidence as studies are based on small numbers of patients and are often uncontrolled.
Ongoing clinical trials seek to:
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
Some current clinical trials also are posted on the following page on the NORD website: https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, in the main, contact: www.centerwatch.com
For more information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
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INTERNET
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NORD y la Fundación MedicAlert se han asociado en un nuevo programa para brindar protección a pacientes con enfermedades raras en situaciones de emergencia.
Aprende más https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/Asegurarse de que los pacientes y los cuidadores estén equipados con las herramientas que necesitan para vivir su mejor vida mientras manejan su condición rara es una parte vital de la misión de NORD.
Aprende más https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/Este programa de asistencia, primero en su tipo, está diseñado para los cuidadores de un niño o adulto diagnosticado con un trastorno raro.
Aprende más https://rarediseases.org/patient-assistance-programs/caregiver-respite/