Stevens-Johnson syndrome
Encyclopedia
Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis
(TEN) are two forms of a life-threatening skin
condition, in which cell death causes the epidermis to separate from the dermis
. The syndrome is thought to be a hypersensitivity
complex that affects the skin and the mucous membrane
s. Although the majority of cases are idiopathic
(without a known cause), the main class of known causes is medication, followed by infections and, rarely, cancers.
(TEN). These conditions were first recognised in 1922.
Both diseases can be mistaken for erythema multiforme
. Erythema multiforme is sometimes caused by a reaction to a medication but is more often a type III hypersensitivity
reaction to an infection (caused most often by Herpes simplex
) and is relatively benign. Although both SJS and TEN can also be caused by infections, they are most often adverse effects of medications. Their consequences are potentially more dangerous than those of erythema multiforme.
SJS usually begins with fever, sore throat, and fatigue, which is misdiagnosed and usually treated with antibiotics. Ulcers and other lesions begin to appear in the mucous membranes, almost always in the mouth and lips but also in the genital and anal regions. Those in the mouth are usually extremely painful and reduce the patient's ability to eat or drink. Conjunctivitis
of the eyes occurs in about 30% of children who develop SJS. A rash of round lesions about an inch across arises on the face, trunk, arms and legs, and soles of the feet, but usually not the scalp.
and erythema multiforme
, are characterized by confluent epidermal necrosis
with minimal associated inflammation. The acuity is apparent from the (normal) basket weave-like pattern of the stratum corneum
.
, influenza
, mumps
, cat-scratch fever, histoplasmosis
, Epstein-Barr virus
, mycoplasma pneumoniae
, or similar.
s and sulfa drugs.
SJS can be caused by adverse effect
s of drugs allopurinol
(a.k.a. Aloprim), Zyloprim, Dilantin, Depakote, Levaquin, diclofenac
, etravirine
, isotretinoin
(a.k.a. Accutane), fluconazole
, valdecoxib
, sitagliptin
, oseltamivir
, penicillin
s, barbiturate
s, sulfonamides
, phenytoin
, azithromycin
, oxcarbazepine
, zonisamide
, modafinil
, lamotrigine
, nevirapine, pyrimethamine
, ibuprofen
, ethosuximide
, carbamazepine
, nystatin
, and gout
medications.
Medications that have traditionally been known to lead to SJS, erythema multiforme and toxic epidermal necrolysis include sulfonamide
s (antibiotics), penicillin
s (antibiotics), barbiturate
s (sedatives), lamotrigine
, and phenytoin
(e.g. Dilantin) (anticonvulsant
s). Combining lamotrigine with sodium valproate
increases the risk of SJS.
Non-steroidal anti-inflammatory drugs are a rare cause of SJS in adults; the risk is higher for older patients, women and those initiating treatment. Typically, the symptoms of drug-induced SJS arise within a week of starting the medication. People with systemic lupus erythematosus
or HIV
infections are more susceptible to drug-induced SJS.
SJS may also be caused by cocaine
use.
populations studied (Han Chinese
and Thai
), carbamazepine
- and phenytoin
-induced SJS is strongly associated with HLA-B*1502 (HLA-B75
), an HLA-B
serotype
of the broader serotype HLA-B15
. A study in Europe suggested that the gene marker
is only relevant for East Asians.
Based on the Asian findings, similar studies in Europe showed 61% of allopurinol
-induced SJS/TEN patients carried the HLA-B58
(phenotype
frequency of the B*5801 allele in Europeans is typically 3%). One study concluded: "Even when HLA-B alleles behave as strong risk factors, as for allopurinol, they are neither sufficient nor necessary to explain the disease."
infections can be treated with oral macrolide
or oral doxycycline
.
Initially, treatment is similar to that for patients with thermal burns, and continued care can only be supportive (e.g. intravenous fluids
and nasogastric
or parenteral feeding) and symptomatic (e.g., analgesic
mouth rinse for mouth ulcer). Dermatologists
and surgeons tend to disagree about whether the skin should be debrided
.
Beyond this kind of supportive care, there is no accepted treatment for SJS. Treatment with corticosteroid
s is controversial. Early retrospective studies suggested that corticosteroids increased hospital stays and complication rates. There are no randomized trials of corticosteroids for SJS, and it can be managed successfully without them.
Other agents have been used, including cyclophosphamide
and cyclosporine, but none has exhibited much therapeutic success. Intravenous immunoglobulin
(IVIG) treatment has shown some promise in reducing the length of the reaction and improving symptoms. Other common supportive measures include the use of topical pain anesthetics and antiseptic
s, maintaining a warm environment, and intravenous analgesics. An ophthalmologist should be consulted immediately, as SJS frequently causes the formation of scar tissue inside the eyelids, leading to corneal vascularization, impaired vision and a host of other ocular problems.
of around 5%. The risk for death can be estimated using the SCORTEN scale
, which takes a number of prognostic indicators into account. Other outcomes include organ damage/failure, cornea scratching, and blindness.
Toxic epidermal necrolysis
Toxic epidermal necrolysis is a rare, life-threatening dermatological condition that is usually induced by a reaction to medications...
(TEN) are two forms of a life-threatening skin
Human skin
The human skin is the outer covering of the body. In humans, it is the largest organ of the integumentary system. The skin has multiple layers of ectodermal tissue and guards the underlying muscles, bones, ligaments and internal organs. Human skin is similar to that of most other mammals,...
condition, in which cell death causes the epidermis to separate from the dermis
Dermis
The dermis is a layer of skin between the epidermis and subcutaneous tissues, and is composed of two layers, the papillary and reticular dermis...
. The syndrome is thought to be a hypersensitivity
Hypersensitivity
Hypersensitivity refers to undesirable reactions produced by the normal immune system, including allergies and autoimmunity. These reactions may be damaging, uncomfortable, or occasionally fatal. Hypersensitivity reactions require a pre-sensitized state of the host. The four-group classification...
complex that affects the skin and the mucous membrane
Mucous membrane
The mucous membranes are linings of mostly endodermal origin, covered in epithelium, which are involved in absorption and secretion. They line cavities that are exposed to the external environment and internal organs...
s. Although the majority of cases are idiopathic
Idiopathic
Idiopathic is an adjective used primarily in medicine meaning arising spontaneously or from an obscure or unknown cause. From Greek ἴδιος, idios + πάθος, pathos , it means approximately "a disease of its own kind". It is technically a term from nosology, the classification of disease...
(without a known cause), the main class of known causes is medication, followed by infections and, rarely, cancers.
Classification
There is agreement in the medical literature that Stevens–Johnson syndrome (SJS) can be considered a milder form of toxic epidermal necrolysisToxic epidermal necrolysis
Toxic epidermal necrolysis is a rare, life-threatening dermatological condition that is usually induced by a reaction to medications...
(TEN). These conditions were first recognised in 1922.
Both diseases can be mistaken for erythema multiforme
Erythema multiforme
Erythema multiforme is a skin condition of unknown cause, possibly mediated by deposition of immune complex in the superficial microvasculature of the skin and oral mucous membrane that usually follows an infection or drug exposure...
. Erythema multiforme is sometimes caused by a reaction to a medication but is more often a type III hypersensitivity
Type III hypersensitivity
Type III hypersensitivity occurs when antigens and antibodies are present in roughly equal amounts, causing extensive cross-linking.-Presentation:...
reaction to an infection (caused most often by Herpes simplex
Herpes simplex
Herpes simplex is a viral disease caused by both Herpes simplex virus type 1 and type 2 . Infection with the herpes virus is categorized into one of several distinct disorders based on the site of infection. Oral herpes, the visible symptoms of which are colloquially called cold sores or fever...
) and is relatively benign. Although both SJS and TEN can also be caused by infections, they are most often adverse effects of medications. Their consequences are potentially more dangerous than those of erythema multiforme.
Signs and symptoms
Conjunctivitis
Conjunctivitis refers to inflammation of the conjunctiva...
of the eyes occurs in about 30% of children who develop SJS. A rash of round lesions about an inch across arises on the face, trunk, arms and legs, and soles of the feet, but usually not the scalp.
Pathology
SJS, like toxic epidermal necrolysisToxic epidermal necrolysis
Toxic epidermal necrolysis is a rare, life-threatening dermatological condition that is usually induced by a reaction to medications...
and erythema multiforme
Erythema multiforme
Erythema multiforme is a skin condition of unknown cause, possibly mediated by deposition of immune complex in the superficial microvasculature of the skin and oral mucous membrane that usually follows an infection or drug exposure...
, are characterized by confluent epidermal necrosis
Necrosis
Necrosis is the premature death of cells in living tissue. Necrosis is caused by factors external to the cell or tissue, such as infection, toxins, or trauma. This is in contrast to apoptosis, which is a naturally occurring cause of cellular death...
with minimal associated inflammation. The acuity is apparent from the (normal) basket weave-like pattern of the stratum corneum
Stratum corneum
The stratum corneum is the outermost layer of the epidermis, consisting of dead cells that lack nuclei and organelles. The purpose of the stratum corneum is to form a barrier to protect underlying tissue from infection, dehydration, chemicals and mechanical stress...
.
Causes
SJS is thought to arise from a disorder of the immune system. The immune reaction can be triggered by infections, drugs, or medications. In some groups, drug reaction may be aggravated by genetic factors.Infections
SJS can be caused by infections. It usually follows common infections such as herpes simplex virusHerpes simplex virus
Herpes simplex virus 1 and 2 , also known as Human herpes virus 1 and 2 , are two members of the herpes virus family, Herpesviridae, that infect humans. Both HSV-1 and HSV-2 are ubiquitous and contagious...
, influenza
Influenza
Influenza, commonly referred to as the flu, is an infectious disease caused by RNA viruses of the family Orthomyxoviridae , that affects birds and mammals...
, mumps
Mumps
Mumps is a viral disease of the human species, caused by the mumps virus. Before the development of vaccination and the introduction of a vaccine, it was a common childhood disease worldwide...
, cat-scratch fever, histoplasmosis
Histoplasmosis
Histoplasmosis is a disease caused by the fungus Histoplasma capsulatum. Symptoms of this infection vary greatly, but the disease primarily affects the lungs...
, Epstein-Barr virus
Epstein-Barr virus
The Epstein–Barr virus , also called human herpesvirus 4 , is a virus of the herpes family and is one of the most common viruses in humans. It is best known as the cause of infectious mononucleosis...
, mycoplasma pneumoniae
Mycoplasma pneumoniae
Mycoplasma pneumoniae is a very small bacterium in the class Mollicutes.It causes the disease mycoplasma pneumonia, a form of atypical bacterial pneumonia, and is related to cold agglutinin disease.-Cell wall/Treatment:...
, or similar.
Medication/drugs
Although Stevens–Johnson Syndrome can be caused by viral infections, malignancies, or severe allergic reactions to medication, the leading cause appears to be the use of antibioticAntibiotic
An antibacterial is a compound or substance that kills or slows down the growth of bacteria.The term is often used synonymously with the term antibiotic; today, however, with increased knowledge of the causative agents of various infectious diseases, antibiotic has come to denote a broader range of...
s and sulfa drugs.
SJS can be caused by adverse effect
Adverse effect (medicine)
In medicine, an adverse effect is a harmful and undesired effect resulting from a medication or other intervention such as surgery.An adverse effect may be termed a "side effect", when judged to be secondary to a main or therapeutic effect. If it results from an unsuitable or incorrect dosage or...
s of drugs allopurinol
Allopurinol
Allopurinol is a drug used primarily to treat hyperuricemia and its complications, including chronic gout.- Mechanism of action :...
(a.k.a. Aloprim), Zyloprim, Dilantin, Depakote, Levaquin, diclofenac
Diclofenac
Diclofenac is a non-steroidal anti-inflammatory drug taken to reduce inflammation and as an analgesic reducing pain in certain conditions....
, etravirine
Etravirine
Etravirine is a drug used for the treatment of HIV. Etravirine is a non-nucleoside reverse transcriptase inhibitor . Unlike the currently available agents in the class, resistance to other NNRTIs does not seem to confer resistance to etravirine. Etravirine is marketed by Tibotec, a subsidiary...
, isotretinoin
Isotretinoin
Isotretinoin, INN, is a medication used mostly for cystic acne. It was first developed for brain, pancreatic and other cancers. It is used to treat harlequin-type ichthyosis, a usually lethal skin disease, and lamellar ichthyosis. Its effects are systemic and nonselective...
(a.k.a. Accutane), fluconazole
Fluconazole
Fluconazole is a triazole antifungal drug used in the treatment and prevention of superficial and systemic fungal infections. In a bulk powder form, it appears as a white crystalline powder, and it is very slightly soluble in water and soluble in alcohol. It is commonly marketed under the trade...
, valdecoxib
Valdecoxib
Valdecoxib is a non-steroidal anti-inflammatory drug used in the treatment of osteoarthritis, rheumatoid arthritis, and painful menstruation and menstrual symptoms. It is a cyclooxygenase-2 selective inhibitor....
, sitagliptin
Sitagliptin
Sitagliptin is an oral antihyperglycemic of the dipeptidyl peptidase-4 inhibitor class. It was developed, and is marketed, by Merck & Co...
, oseltamivir
Oseltamivir
Oseltamivir INN , an antiviral drug, slows the spread of influenza virus between cells in the body by stopping the virus from chemically cutting ties with its host cell; median time to symptom alleviation is reduced by 0.5–1 day. The drug is sold under the trade name Tamiflu, and is taken orally...
, penicillin
Penicillin
Penicillin is a group of antibiotics derived from Penicillium fungi. They include penicillin G, procaine penicillin, benzathine penicillin, and penicillin V....
s, barbiturate
Barbiturate
Barbiturates are drugs that act as central nervous system depressants, and can therefore produce a wide spectrum of effects, from mild sedation to total anesthesia. They are also effective as anxiolytics, as hypnotics, and as anticonvulsants...
s, sulfonamides
Sulfonamide (medicine)
Sulfonamide or sulphonamide is the basis of several groups of drugs. The original antibacterial sulfonamides are synthetic antimicrobial agents that contain the sulfonamide group. Some sulfonamides are also devoid of antibacterial activity, e.g., the anticonvulsant sultiame...
, phenytoin
Phenytoin
Phenytoin sodium is a commonly used antiepileptic. Phenytoin acts to suppress the abnormal brain activity seen in seizure by reducing electrical conductance among brain cells by stabilizing the inactive state of voltage-gated sodium channels...
, azithromycin
Azithromycin
Azithromycin is an azalide, a subclass of macrolide antibiotics. Azithromycin is one of the world's best-selling antibiotics...
, oxcarbazepine
Oxcarbazepine
Oxcarbazepine is a anticholinergic anticonvulsant and mood stabilizing drug, used primarily in the treatment of epilepsy. It is also used to treat anxiety and mood disorders, and benign motor tics...
, zonisamide
Zonisamide
Zonisamide is a sulfonamide anticonvulsant approved for use as an adjunctive therapy in adults with partial-onset seizures for adults; infantile spasm, mixed seizure types of Lennox-Gastaut syndrome, myoclonic, and generalized tonic clonic seizure.-History:...
, modafinil
Modafinil
Modafinil is an analeptic drug manufactured by Cephalon, and is approved by the U.S. Food and Drug Administration for the treatment of narcolepsy, shift work sleep disorder, and excessive daytime sleepiness associated with obstructive sleep apnea...
, lamotrigine
Lamotrigine
Lamotrigine, marketed in the US and most of Europe as Lamictal by GlaxoSmithKline, is an anticonvulsant drug used in the treatment of epilepsy and bipolar disorder. It is also used as an adjunct in treating depression, though this is considered off-label usage...
, nevirapine, pyrimethamine
Pyrimethamine
Pyrimethamine is a medication used for protozoal infections. It is commonly used as an antimalarial drug , and is also used in the treatment of Toxoplasma gondii infections in immunocompromised patients, such as HIV-positive individuals.-Mechanism of action:Pyrimethamine interferes with...
, ibuprofen
Ibuprofen
Ibuprofen is a nonsteroidal anti-inflammatory drug used for relief of symptoms of arthritis, fever, as an analgesic , especially where there is an inflammatory component, and dysmenorrhea....
, ethosuximide
Ethosuximide
Ethosuximide is a succinimide anticonvulsant, used mainly in absence seizures.-Approved:It is approved for absence seizures. Ethosuximide is considered the first choice drug for treating absence seizures in part because it lacks the idiosyncratic hepatotoxicity of the alternative anti-absence...
, carbamazepine
Carbamazepine
Carbamazepine is an anticonvulsant and mood-stabilizing drug used primarily in the treatment of epilepsy and bipolar disorder, as well as trigeminal neuralgia...
, nystatin
Nystatin
Nystatin is a polyene antifungal medication to which many molds and yeast infections are sensitive, including Candida. Due to its toxicity profile, there are currently no injectable formulations of this drug on the US market...
, and gout
Gout
Gout is a medical condition usually characterized by recurrent attacks of acute inflammatory arthritis—a red, tender, hot, swollen joint. The metatarsal-phalangeal joint at the base of the big toe is the most commonly affected . However, it may also present as tophi, kidney stones, or urate...
medications.
Medications that have traditionally been known to lead to SJS, erythema multiforme and toxic epidermal necrolysis include sulfonamide
Sulfonamide (medicine)
Sulfonamide or sulphonamide is the basis of several groups of drugs. The original antibacterial sulfonamides are synthetic antimicrobial agents that contain the sulfonamide group. Some sulfonamides are also devoid of antibacterial activity, e.g., the anticonvulsant sultiame...
s (antibiotics), penicillin
Penicillin
Penicillin is a group of antibiotics derived from Penicillium fungi. They include penicillin G, procaine penicillin, benzathine penicillin, and penicillin V....
s (antibiotics), barbiturate
Barbiturate
Barbiturates are drugs that act as central nervous system depressants, and can therefore produce a wide spectrum of effects, from mild sedation to total anesthesia. They are also effective as anxiolytics, as hypnotics, and as anticonvulsants...
s (sedatives), lamotrigine
Lamotrigine
Lamotrigine, marketed in the US and most of Europe as Lamictal by GlaxoSmithKline, is an anticonvulsant drug used in the treatment of epilepsy and bipolar disorder. It is also used as an adjunct in treating depression, though this is considered off-label usage...
, and phenytoin
Phenytoin
Phenytoin sodium is a commonly used antiepileptic. Phenytoin acts to suppress the abnormal brain activity seen in seizure by reducing electrical conductance among brain cells by stabilizing the inactive state of voltage-gated sodium channels...
(e.g. Dilantin) (anticonvulsant
Anticonvulsant
The anticonvulsants are a diverse group of pharmaceuticals used in the treatment of epileptic seizures. Anticonvulsants are also increasingly being used in the treatment of bipolar disorder, since many seem to act as mood stabilizers, and in the treatment of neuropathic pain. The goal of an...
s). Combining lamotrigine with sodium valproate
Sodium valproate
Sodium valproate or valproate sodium is the sodium salt of valproic acid and is an anticonvulsant used in the treatment of epilepsy, anorexia nervosa, panic attack, anxiety disorder, posttraumatic stress disorder, migraine and bipolar disorder, as well as other psychiatric conditions requiring...
increases the risk of SJS.
Non-steroidal anti-inflammatory drugs are a rare cause of SJS in adults; the risk is higher for older patients, women and those initiating treatment. Typically, the symptoms of drug-induced SJS arise within a week of starting the medication. People with systemic lupus erythematosus
Systemic lupus erythematosus
Systemic lupus erythematosus , often abbreviated to SLE or lupus, is a systemic autoimmune disease that can affect any part of the body. As occurs in other autoimmune diseases, the immune system attacks the body's cells and tissue, resulting in inflammation and tissue damage...
or HIV
HIV
Human immunodeficiency virus is a lentivirus that causes acquired immunodeficiency syndrome , a condition in humans in which progressive failure of the immune system allows life-threatening opportunistic infections and cancers to thrive...
infections are more susceptible to drug-induced SJS.
SJS may also be caused by cocaine
Cocaine
Cocaine is a crystalline tropane alkaloid that is obtained from the leaves of the coca plant. The name comes from "coca" in addition to the alkaloid suffix -ine, forming cocaine. It is a stimulant of the central nervous system, an appetite suppressant, and a topical anesthetic...
use.
Genetics
In some East AsianEthnic groups in Asia
There are an abundance of ethnic groups throughout Asia, with adaptations to the climate zones of Asia, which can be Arctic, subarctic, temperate, subtropical or tropical. The ethnic groups have adapted to mountains, deserts, grasslands, and forests. On the coasts of Asia, the ethnic groups have...
populations studied (Han Chinese
Han Chinese
Han Chinese are an ethnic group native to China and are the largest single ethnic group in the world.Han Chinese constitute about 92% of the population of the People's Republic of China , 98% of the population of the Republic of China , 78% of the population of Singapore, and about 20% of the...
and Thai
Thai people
The Thai people, or Siamese, are the main ethnic group of Thailand and are part of the larger Tai ethnolinguistic peoples found in Thailand and adjacent countries in Southeast Asia as well as southern China. Their language is the Thai language, which is classified as part of the Kradai family of...
), carbamazepine
Carbamazepine
Carbamazepine is an anticonvulsant and mood-stabilizing drug used primarily in the treatment of epilepsy and bipolar disorder, as well as trigeminal neuralgia...
- and phenytoin
Phenytoin
Phenytoin sodium is a commonly used antiepileptic. Phenytoin acts to suppress the abnormal brain activity seen in seizure by reducing electrical conductance among brain cells by stabilizing the inactive state of voltage-gated sodium channels...
-induced SJS is strongly associated with HLA-B*1502 (HLA-B75
HLA-B75
HLA-B75 is an HLA-B serotype. The serotype identifies certain B*15 gene-allele protein products of HLA-B.B75 is one of many split antigens of the broad antigen, B15...
), an HLA-B
HLA-B
HLA-B is a human gene that provides instructions for making a protein that plays a critical role in the immune system. HLA-B is part of a family of genes called the human leukocyte antigen complex...
serotype
Serotype
Serotype or serovar refers to distinct variations within a subspecies of bacteria or viruses. These microorganisms, viruses, or cells are classified together based on their cell surface antigens...
of the broader serotype HLA-B15
HLA-B15
HLA-B15 is an HLA-B serotype. The serotype identifies the B*15 gene-allele protein products of HLA-B.B15 is a broad antigen can be subdivided into several split antigens that are often used in characterization. These are B62, B63, B70, B71, B72, B75, B76, B77...
. A study in Europe suggested that the gene marker
Genetic marker
A genetic marker is a gene or DNA sequence with a known location on a chromosome that can be used to identify cells, individuals or species. It can be described as a variation that can be observed...
is only relevant for East Asians.
Based on the Asian findings, similar studies in Europe showed 61% of allopurinol
Allopurinol
Allopurinol is a drug used primarily to treat hyperuricemia and its complications, including chronic gout.- Mechanism of action :...
-induced SJS/TEN patients carried the HLA-B58
HLA-B58
HLA-B58 is an HLA-B serotype. B58 is a split antigen from the B17 broad antigen, the sister serotype B57. The serotype identifies the more common HLA-B*58 gene products...
(phenotype
Phenotype
A phenotype is an organism's observable characteristics or traits: such as its morphology, development, biochemical or physiological properties, behavior, and products of behavior...
frequency of the B*5801 allele in Europeans is typically 3%). One study concluded: "Even when HLA-B alleles behave as strong risk factors, as for allopurinol, they are neither sufficient nor necessary to explain the disease."
Treatment
SJS constitutes a dermatological emergency. All medications should be discontinued, particularly those known to cause SJS reactions. Patients with documented mycoplasmaMycoplasma
Mycoplasma refers to a genus of bacteria that lack a cell wall. Without a cell wall, they are unaffected by many common antibiotics such as penicillin or other beta-lactam antibiotics that target cell wall synthesis. They can be parasitic or saprotrophic. Several species are pathogenic in humans,...
infections can be treated with oral macrolide
Macrolide
The macrolides are a group of drugs whose activity stems from the presence of a macrolide ring, a large macrocyclic lactone ring to which one or more deoxy sugars, usually cladinose and desosamine, may be attached. The lactone rings are usually 14-, 15-, or 16-membered...
or oral doxycycline
Doxycycline
Doxycycline INN is a member of the tetracycline antibiotics group, and is commonly used to treat a variety of infections. Doxycycline is a semisynthetic tetracycline invented and clinically developed in the early 1960s by Pfizer Inc. and marketed under the brand name Vibramycin. Vibramycin...
.
Initially, treatment is similar to that for patients with thermal burns, and continued care can only be supportive (e.g. intravenous fluids
Intravenous therapy
Intravenous therapy or IV therapy is the infusion of liquid substances directly into a vein. The word intravenous simply means "within a vein". Therapies administered intravenously are often called specialty pharmaceuticals...
and nasogastric
Nasogastric intubation
Nasogastric intubation is a medical process involving the insertion of a plastic tube through the nose, past the throat, and down into the stomach.-Uses:...
or parenteral feeding) and symptomatic (e.g., analgesic
Analgesic
An analgesic is any member of the group of drugs used to relieve pain . The word analgesic derives from Greek an- and algos ....
mouth rinse for mouth ulcer). Dermatologists
Dermatology
Dermatology is the branch of medicine dealing with the skin and its diseases, a unique specialty with both medical and surgical aspects. A dermatologist takes care of diseases, in the widest sense, and some cosmetic problems of the skin, scalp, hair, and nails....
and surgeons tend to disagree about whether the skin should be debrided
Debridement
Debridement is the medical removal of a patient's dead, damaged, or infected tissue to improve the healing potential of the remaining healthy tissue...
.
Beyond this kind of supportive care, there is no accepted treatment for SJS. Treatment with corticosteroid
Corticosteroid
Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex. Corticosteroids are involved in a wide range of physiologic systems such as stress response, immune response and regulation of inflammation, carbohydrate metabolism, protein catabolism, blood electrolyte...
s is controversial. Early retrospective studies suggested that corticosteroids increased hospital stays and complication rates. There are no randomized trials of corticosteroids for SJS, and it can be managed successfully without them.
Other agents have been used, including cyclophosphamide
Cyclophosphamide
Cyclophosphamide , also known as cytophosphane, is a nitrogen mustard alkylating agent, from the oxazophorines group....
and cyclosporine, but none has exhibited much therapeutic success. Intravenous immunoglobulin
Intravenous immunoglobulin
Intravenous immunoglobulin is a blood product administered intravenously. It contains the pooled IgG extracted from the plasma of over one thousand blood donors. IVIG's effects last between 2 weeks and 3 months...
(IVIG) treatment has shown some promise in reducing the length of the reaction and improving symptoms. Other common supportive measures include the use of topical pain anesthetics and antiseptic
Antiseptic
Antiseptics are antimicrobial substances that are applied to living tissue/skin to reduce the possibility of infection, sepsis, or putrefaction...
s, maintaining a warm environment, and intravenous analgesics. An ophthalmologist should be consulted immediately, as SJS frequently causes the formation of scar tissue inside the eyelids, leading to corneal vascularization, impaired vision and a host of other ocular problems.
Prognosis
SJS proper (with less than 10% of body surface area involved) has a mortality rateMortality rate
Mortality rate is a measure of the number of deaths in a population, scaled to the size of that population, per unit time...
of around 5%. The risk for death can be estimated using the SCORTEN scale
SCORTEN scale
The SCORTEN Scale is a severity-of-illness scale with which the severity of certain bullous conditions can be systematically determined. It was originally developed for Toxic Epidermal Necrolysis, but can be used with burn victims, sufferers of Stevens-Johnson Syndrome, cutaneous drug reactions, or...
, which takes a number of prognostic indicators into account. Other outcomes include organ damage/failure, cornea scratching, and blindness.
Epidemiology
Stevens–Johnson syndrome is a rare condition, with a reported incidence of around 2.6 to 6.1 cases per million people per year. In the United States, there are about 300 new diagnoses per year. The condition is more common in adults than in children. Women are affected more often than men, with cases occurring at a two to one (2:1) ratio.History
Stevens-Johnson Syndrome is named for Albert Mason Stevens and Frank Chambliss Johnson, American pediatricians who in 1922 jointly published a description of the disorder in the American Journal of Diseases of Children.Notable cases
- Padma LakshmiPadma LakshmiPadma Parvati Lakshmi also Padma, Lady Rushdie is an American cookbook author, actress, model and television host. She has been the host of the US reality television program Top Chef since season two in 2006, for which she received a nomination for the Primetime Emmy Award for Outstanding Host for...
, actress, model, television personality, and cookbook writer; - Tessa Keller of MTV show Laguna BeachLaguna Beach: The Real Orange CountyLaguna Beach: The Real Orange County, often referred to simply as Laguna Beach, is a reality television series which originally aired on MTV from September 28, 2004 until November 16, 2006. It documents the lives of several teenagers living in Laguna Beach, an affluent seaside community located in...
; - Sabrina Brierton Johnson, whose family unsuccessfully sued the manufacturer of Children's Motrin, Johnson & JohnsonJohnson & JohnsonJohnson & Johnson is an American multinational pharmaceutical, medical devices and consumer packaged goods manufacturer founded in 1886. Its common stock is a component of the Dow Jones Industrial Average and the company is listed among the Fortune 500....
, after a case of SJS blinded her. - Manute BolManute BolManute Bol was a Sudanese-born basketball player and activist. At 7 feet, 7 inches , Bol was one of the tallest players ever to appear in the National Basketball Association, along with Gheorghe Mureşan. Unlike Mureşan, however, Bol was naturally tall and did not have a Pituitary disease...
, former professional basketball player and member of NBA's Washington Bullets, Golden State WarriorsGolden State WarriorsThe Golden State Warriors are an American professional basketball team based in Oakland, California. They are part of the Pacific Division of the Western Conference in the National Basketball Association...
, Philadelphia 76ersPhiladelphia 76ersThe Philadelphia 76ers are a professional basketball team based in Philadelphia, Pennsylvania. They play in the Atlantic Division of the Eastern Conference of the National Basketball Association . Originally known as the Syracuse Nationals, they are one of the oldest franchises in the NBA...
, and Miami HeatMiami HeatThe Miami Heat is a professional basketball team based in Miami, Florida, United States. The team is a member of the Southeast Division in the Eastern Conference of the National Basketball Association . They play their home games at American Airlines Arena in Downtown Miami...
, who died from complications. - On the television show HouseHouse (TV series)House is an American television medical drama that debuted on the Fox network on November 16, 2004. The show's central character is Dr. Gregory House , an unconventional and misanthropic medical genius who heads a team of diagnosticians at the fictional Princeton-Plainsboro Teaching Hospital in...
(The Confession, season 8, episode 5), the patient was suspected of having Stevens-Johnson syndrome.