Materiały źródłowe

• #1 Stevens-Johnson syndrome and toxic epidermal necrolysis: Pathogenesis, clinical manifestations, and diagnosis – UpToDate

  https://www.uptodate.com/contents/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-pathogenesis-clinical-manifestations-and-diagnosis

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions characterized by extensive necrosis and detachment of the epidermis. Mucous membranes are affected in more than 90 percent of patients, usually at two or more distinct sites. […] SJS and TEN exist as a continuum and are classified based on the percentage of skin body surface area (BSA) detached: […] <10 percent of BSA detached – SJS [...] 10 to 30 percent of BSA detached – SJS/TEN overlap [...] >30 percent of BSA detached – TEN.

• #2 Stevens-Johnson syndrome and toxic epidermal necrolysis: Management, prognosis, and long-term sequelae – UpToDate

  https://www.uptodate.com/contents/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-management-prognosis-and-long-term-sequelae/print

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe, mucocutaneous, adverse reactions in most cases induced by medications. Clinical features include fever, prodromal symptoms, mucositis, and extensive epidermal detachment. SJS and TEN are considered a disease continuum and are collectively known as epidermal necrolysis. […] SJS is defined as skin detachment affecting <10 percent of the BSA. [...] TEN is defined as skin detachment affecting >30 percent of the BSA. […] SJS/TEN overlap is defined as skin detachment involving 10 to 30 percent of the BSA.

• #3 Stevens-Johnson syndrome and toxic epidermal necrolysis – Symptoms, diagnosis and treatment | BMJ Best Practice

  https://bestpractice.bmj.com/topics/en-gb/237

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe skin detachments with mucocutaneous complications associated with a history of medication use, most commonly anticonvulsants, antibiotics, and non-steroidal anti-inflammatory drugs. […] SJS/TEN may present with mild symptoms in early disease, but the condition progresses over several days; close monitoring and regular reassessment is essential. […] Classification is dependent on the percentage of skin involvement: SJS has 10% total body surface area (TBSA) involvement; SJS/TEN overlap has 10% to 30% TBSA involvement; and TEN has 30% TBSA involvement.

• #4 Stevens-Johnson syndrome – Symptoms & causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/stevens-johnson-syndrome/symptoms-causes/syc-20355936

  Stevens-Johnson syndrome (SJS) is a rare, serious disorder of the skin and mucous membranes. It’s usually a reaction to medication that starts with flu-like symptoms, followed by a painful rash that spreads and blisters. Then the top layer of affected skin dies, sheds and begins to heal after several days. […] One to three days before a rash develops, you may show early signs of Stevens-Johnson syndrome, including: Fever, A sore mouth and throat, Fatigue, Burning eyes. […] As the condition develops, other signs and symptoms include: Unexplained widespread skin pain, A red or purple rash that spreads, Blisters on your skin and the mucous membranes of the mouth, nose, eyes and genitals, Shedding of skin within days after blisters form. […] Stevens-Johnson syndrome requires immediate medical attention. Seek emergency medical care if you experience signs and symptoms of this condition. Drug-induced reactions may occur during the use of a medication or up to two weeks after discontinuing it.

• #5 Stevens-Johnson Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459323/

  Stevens-Johnson syndrome/toxic epidermal necrolysis is a rare, acute, serious, and potentially fatal skin reaction in which there are sheet-like skin and mucosal loss accompanied by systemic symptoms. Medications are causative in over 80% of cases. […] The illness begins with nonspecific symptoms such as fever and malaise, upper respiratory tract symptoms such as a cough, rhinitis, sore eyes, and myalgia. Over the next three to four days, a blistering rash and erosions appear on the face, trunk, limbs, and mucosal surfaces. […] Early on, toxic epidermal necrolysis displays widespread tender erythroderma and erosions (with or without targetoid rash), whereas Stevens-Johnson syndrome is characterized more by targetoid rash, with fewer areas of denudation. […] The patient is very ill, anxious, and in pain. Liver, kidneys, lungs, bone marrow, and joints may be affected by Stevens-Johnson syndrome/toxic epidermal necrolysis. Typical symptoms include: Fever, malaise, headache, anorexia, pharyngitis.

• #5 Stevens-Johnson Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK459323/

  The severity of Stevens-Johnson syndrome/toxic epidermal necrolysis is assessed using SCORTEN. One point is scored for each of the following seven criteria at admission. […] The risk of dying from Stevens-Johnson syndrome/toxic epidermal necrolysis depends on the score. The mortality rate is more than 40 times higher in those with bicarbonate levels less than 20 mmol/L compared with those with higher levels. […] The outcomes of patients with SJS depend on the extent and severity of skin involvement. For those with a mild eruption, the lesions usually heal in 12 to 16 weeks. Mild scarring may occur, but there is usually no functional loss unless the eyes and other mucous membranes are involved. When the involved skin area is more than 20%, mortality rates of 1 to 27% have been reported. […] Survivors of SJS may develop inverted eyelids, sicca-like syndrome, visual loss, and corneal neovascularization, but an interprofessional approach will lead to better outcomes.

• #6 Stevens Johnson Syndrome / Toxic Epidermal Necrolysis. SJS/TEN

  https://dermnetnz.org/topics/stevens-johnson-syndrome-toxic-epidermal-necrolysis

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are now believed to be variants of the same condition, distinct from erythema multiforme. SJS/TEN is a rare, acute, serious, and potentially fatal skin reaction in which there are sheet-like skin and mucosal loss. […] SJS/TEN usually develops within the first week of antibiotic therapy but up to 2 months after starting an anticonvulsant. For most drugs, the onset is within a few days up to 1 month. […] Before the rash appears, there is usually a prodromal illness of several days duration resembling an upper respiratory tract infection or 'flu-like illness. Symptoms may include: Fever 39 C, Sore throat, difficulty swallowing, Runny nose and cough, Sore red eyes, conjunctivitis, General aches and pains. […] There is then an abrupt onset of a tender/painful red skin rash starting on the trunk and extending rapidly over hours to days onto the face and limbs (but rarely affecting the scalp, palms or soles). The maximum extent is usually reached by four days.

• #6 Stevens Johnson Syndrome / Toxic Epidermal Necrolysis. SJS/TEN

  https://dermnetnz.org/topics/stevens-johnson-syndrome-toxic-epidermal-necrolysis

  The skin lesions may be: Macules flat, red and diffuse (measles-like spots) or purple (purpuric) spots, Diffuse erythema, Targetoid as in erythema multiforme, Blisters flaccid (ie, not tense). […] The blisters then merge to form sheets of skin detachment, exposing red, oozing dermis. The Nikolsky sign is positive in areas of skin redness. This means that blisters and erosions appear when the skin is rubbed gently. […] Mucosal involvement is prominent and severe, although not forming actual blisters. At least two mucosal surfaces are affected including: Eyes (conjunctivitis, less often corneal ulceration, anterior uveitis, panophthalmitis) red, sore, sticky, photosensitive eyes, Lips/mouth (cheilitis, stomatitis) red crusted lips, painful mouth ulcers, Pharynx, oesophagus causing difficulty eating, Genital area and urinary tract erosions, ulcers, urinary retention, Upper respiratory tract (trachea and bronchi) cough and respiratory distress, Gastrointestinal tract diarrhoea.

• #6 Stevens Johnson Syndrome / Toxic Epidermal Necrolysis. SJS/TEN

  https://dermnetnz.org/topics/stevens-johnson-syndrome-toxic-epidermal-necrolysis

  The patient is very ill, extremely anxious and in considerable pain. In addition to skin/mucosal involvement, other organs may be affected including liver, kidneys, lungs, bone marrow and joints. […] The acute phase of SJS/TEN lasts 8-12 days. […] It may take weeks to months for symptoms and signs to settle.

• #7 Stevens-Johnson Syndrome Clinical Presentation: History, Physical Examination, Complications

  https://emedicine.medscape.com/article/1197450-clinical

  Typowo zespół Stevensa-Johnsona (SJS) zaczyna się od niespecyficznej infekcji górnych dróg oddechowych. […] Mucocutaneous lesions develop abruptly. Clusters of outbreaks last from 2-4 weeks. The lesions typically are nonpruritic. […] A history of fever or localized worsening should suggest a superimposed infection; however, fever has been reported to occur in up to 85% of cases. […] Involvement of oral and/or mucous membranes may be severe enough that patients may not be able to eat or drink. […] Patients may complain of a burning rash that begins symmetrically on the face and the upper part of the torso. This may be accompanied by ocular symptoms. […] Ocular symptoms include the following: Red eye, Tearing, Dry eye, Pain, Blepharospasm, Itching, Grittiness, Heavy eyelid, Foreign body sensation, Decreased vision, Burning sensation, Photophobia, Diplopia.

• #7 Stevens-Johnson Syndrome Clinical Presentation: History, Physical Examination, Complications

  https://emedicine.medscape.com/article/1197450-clinical

  Of patients with Stevens-Johnson syndrome, 27-50% progress to severe ocular disease. […] Lesions may continue to erupt in crops for as long as 2-3 weeks. Mucosal pseudomembrane formation may lead to mucosal scarring and loss of function of the involved organ system. […] Blindness may develop secondary to severe keratitis or panophthalmitis in 3-10% of patients.

• #8 Stevens-Johnson Syndrome (SJS): Causes, Rash & Treatments

  https://my.clevelandclinic.org/health/diseases/17656-stevens-johnson-syndrome

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious skin conditions that cause your skin to develop rashes, blisters, and then peel. Your mucus membranes, including your eyes, genitalia and mouth, are also affected. If you get this condition, you’ll likely be admitted to a hospital. […] Symptoms of Stevens-Johnson syndrome include: Skin pain. Fever. Body aches. Red rash or red blotches on your skin. Cough. Blisters and sores on the skin and on mucus membranes of the mouth, throat, eyes, genitals and anus. Peeling skin. Drooling (because closing the mouth is painful). Eyes sealed shut (due to blisters and swelling). Painful urination (due to blistered mucus membranes). […] If your case of SJS is caused by a drug, symptoms appear about one to three weeks after you start taking medication. The flu-like illness (fever, cough and headache, skin pain) is followed first by a rash and then peeling. In the case of TEN, some people even lose hair and nails. SJS may also be linked to recent vaccinations, though this is very rare.

• #8 Stevens-Johnson Syndrome (SJS): Causes, Rash & Treatments

  https://my.clevelandclinic.org/health/diseases/17656-stevens-johnson-syndrome

  Each person’s experience with Stevens-Johnson syndrome can be different. Skin can regrow in a matter of weeks, but recovery can take months if symptoms are severe. Some long-term reactions may develop, including: Skin: dryness, itching, change in skin color. Eyes: chronic swelling and/or dryness, chronic irritation, difficulty seeing, sensitivity to light (photophobia). […] SJS results in death in 10% of patients and in 30% for those with TEN due mostly to sepsis, acute respiratory distress syndrome and multiple organ failure.

• #9 Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis | CommonSpirit Health

  https://www.commonspirit.org/conditions-treatments/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis

  Stevens-Johnson syndrome is a rare but serious condition that causes a rash and sores on the body’s mucous membranes, which are moist tissues that line the inside of the body. […] Early symptoms of Stevens-Johnson syndrome may include: Fever. Fatigue, a general feeling of sickness. Itching or burning eyes. Body aches and joint pain. […] Within 1 to 3 days, other symptoms of Stevens-Johnson syndrome appear. These symptoms may include: Weeping sores on the mucous membranes of the mouth, nose, genitals, and eyes. When sores occur, it can be painful to eat, drink, or urinate. A skin rash with red spots. The rash may be painful and peel off. It may happen on any part of the body but often begins on the face and chest (trunk). […] Even mild Stevens-Johnson syndrome can get worse quickly and become serious, so it’s treated in the hospital. Recovery from Stevens-Johnson syndrome may be slow. It may take weeks or months based on how bad the condition is.

• #10 Stevens-Johnson syndrome

  https://www.nhs.uk/conditions/stevens-johnson-syndrome/

  Stevens-Johnson syndrome can start with flu-like symptoms, such as a high temperature, sore throat, cough and joint pain. […] A rash appears a few days later. It’s made up of circular patches that are darker in the middle and lighter around the outside. […] The rash usually starts on the upper body before quickly spreading to the face, arms, legs and other areas of the body, such as the genitals. It’s not usually itchy. […] Blisters then appear on your skin, which burst to leave painful sores. The skin often peels very easily. […] You can also get blisters and sores: on your lips and in your mouth and throat this can make swallowing painful; in the tube that carries pee out of your body this can cause pain when peeing; on your eyes this can cause eye pain, pain when looking at bright lights and problems with your sight.

• #11 Stevens-Johnson Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1197450-overview

  Typical prodromal symptoms of Stevens-Johnson syndrome are as follows: Cough productive of a thick, purulent sputum. Headache. Malaise. Arthralgia. […] Patients may complain of a burning rash that begins symmetrically on the face and the upper part of the torso. The cutaneous lesions are characterized as follows: The rash can begin as macules that develop into papules, vesicles, bullae, urticarial plaques, or confluent erythema. The typical lesion has the appearance of a target; this is considered pathognomonic. In contrast to the typical lesions of erythema multiforme, these lesions have only 2 zones of color. The lesions core may be vesicular, purpuric, or necrotic; that zone is surrounded by macular erythema. Lesions may become bullous and later rupture, leaving denuded skin; the skin becomes susceptible to secondary infection.

• #11 Stevens-Johnson Syndrome: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1197450-overview

  Signs of mucosal involvement can include the following: Erythema. Edema. Sloughing. Blistering. Ulceration. Necrosis. […] Individual lesions typically should heal within 1-2 weeks, unless secondary infection occurs. Most patients recover without sequelae. Mortality is determined primarily by the extent of skin sloughing. When body surface area (BSA) sloughing is less than 10%, the mortality rate is approximately 1-5%. However, when more than 30% BSA sloughing is present, the mortality rate is between 25% and 35%, and may be as high as 50%. […] Although some patients rapidly progress to lose very large areas of the epidermis in a matter of days, the process suddenly ceases in others and re-epithelialization begins a few days later. Predicting the course of disease in a given patient at the initial presentation is not possible. Re-epithelialization is usually complete within 3 weeks, but pressure and mucosal areas may remain eroded and crusted for 2 weeks or longer. Survivors of Stevens-Johnson syndrome may experience numerous long-term sequelae; the most disabling are those of the eye. Cicatrization of conjunctival erosions may lead to the following: Inverted eyelashes. Photophobia. A burning sensation in the eyes. Watery eyes. A keratitis siccalike syndrome. Corneal and conjunctival neovascularization. As many as 40% of survivors of toxic epidermal necrolysis have residual potentially disabling lesions that may cause blindness.

• #12 Lamotrigine and Stevens-Johnson Syndrome Prevention

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8146560/

  The overall mortality for SJS is less than 10% for SJS and between 30 to 50% for TEN. […] Long-term complications can be disabling, particularly ocular complications, and the development of adhesions and strictures throughout the gastrointestinal and urogenital tracts can occur. […] SJS first presents with a prodromal phase occurring within the first few weeks where patients experience fever, malaise, rhinorrhea, photophobia, and erythema. […] After the prodromal period, patients enter a period largely dominated by cutaneous manifestations with development of mucosal and cutaneous lesions easily removed with pressure to the skin. […] In SJS, epidermal detachment involves less than 10% of the body. […] Mucosal lesions primarily involve the oropharynx, conjunctiva, genitals, and the gastrointestinal tract.

• #12 Lamotrigine and Stevens-Johnson Syndrome Prevention

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8146560/

  Lesions are flat and atypical, developing on the trunk and then spreading to the extremities. […] Blood studies of SJS presents with anemia and leukopenia, acting as poor predictors of prognosis. […] Long term complications include changes in pigmentation, skin scarring, visual impairment, periodontal disease, gastrointestinal complications, and vaginal and urethral stenosis. […] The risk of developing SJS increases with infection, malignancy, autoimmune disorders, drug use, and certain HLA phenotypes. […] Hypersensitivity reactions to certain drugs is the leading cause. […] Multiple drugs include but not limited to lamotrigine, nevirapine, phenytoin, and NSAIDs (more commonly celecoxib and ibuprofen). […] Lamotrigine has been the number one drug and carbamazepine as the second associated with SJS.

• #12 Lamotrigine and Stevens-Johnson Syndrome Prevention

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8146560/

  Stevens-Johnson Syndrome (SJS) is a rare life-threatening condition characterized by severe mucocutaneous epidermal necrolysis and detachment of the epidermis. […] Early signs of SJS can be extremely difficult to diagnose, often leading to misdiagnoses and a delay in treatment. Many patients will present with nonspecific prodromal symptoms occurring 1 to 3 weeks after drug introduction. […] Mucosal lesions often present first and can include oral, respiratory, conjunctival, and genitourinary regions. […] These lesions typically begin as diffuse ill-defined erythematous macules with purpuric centers that progress to vesicles and bullae before sloughing off after days. […] Diagnosis is made by skin biopsy with findings of full-thickness dermal necrosis. […] This acute phase of SJS usually lasts between 8-12 days and may result in large, painful areas of denuded skin that will begin to undergo re-epithelialization about 1 week after onset of symptoms and can take up to 3 weeks.

• #13 Stevens-Johnson Syndrome (Causes, Symptoms, and Treatment)

  https://patient.info/doctor/stevens-johnson-syndrome

  Stevens-Johnson syndrome symptoms often starts with a nonspecific upper respiratory tract infection, which may be associated with fever, sore throat, chills, headache, arthralgia, vomiting and diarrhoea, and malaise. […] Mucocutaneous lesions develop suddenly and clusters of outbreaks last from 2-4 weeks. The lesions are usually not pruritic. […] Mouth: severe oromucosal ulceration. […] Respiratory involvement may cause a cough productive of a thick purulent sputum. […] Patients with genitourinary involvement may complain of dysuria or an inability to pass urine. […] Ocular symptoms: painful red eye, purulent conjunctivitis, photophobia, blepharitis. […] Many patients surviving SJS and more that 50% surviving TEN experience long-term sequelae involving the skin, mucous membranes or eyes. These include: […] The overall mortality rate is up to 10% for SJS and at least 30% for TEN. However, the mortality rate correlates with the SCORTEN score and is greater than 90% for people with a SCORTEN score of 5 or more.

• #14 Stevens-Johnson Syndrome Treatment Options and Symptoms – The Kingsley Clinic

  https://thekingsleyclinic.com/resources/stevens-johnson-syndrome-treatment-options-and-symptoms/

  Blisters, a key diagnostic feature, occur in 85-95% of SJS patients. These fluid-filled lesions develop on areas affected by the rash. […] Mucosal lesions affect approximately 90% of SJS patients. These painful sores can appear in the mouth, eyes, genitals, and other mucous membranes. […] A sore throat occurs in 30-50% of cases and can be an early sign of mucosal involvement. […] Cough is reported in 20-40% of SJS patients and may indicate respiratory tract involvement. […] Eye irritation affects 70-85% of SJS patients and is a significant cause of morbidity. […] Skin pain, experienced by 85-95% of SJS patients, often precedes visible skin changes. […] Fatigue is a common symptom, reported in 60-80% of SJS patients. […] Headache occurs in 30-50% of SJS cases and is often associated with fever and systemic inflammation.

• #15

  https://www.healthxchange.sg/head-neck/skin-health/stevens-johnson-syndrome-potentially-deadly-skin-disease

  Stevens-Johnson syndrome is caused by a severe allergic reaction to certain drugs. […] This is a rare and potentially life-threatening skin disease characterised by a red, painful and blistering rash all over the body. […] Often, Stevens-Johnson syndrome begins with non-specific symptoms such as fever, sore throat and irritation in the eyes. These flu-like symptoms are often misdiagnosed by doctors and treated with antibiotics. […] As the condition worsens, more specific symptoms develop: Skin lesions: Red or purplish patchy rash that may appear on the face, arms and legs. It may be symmetrical on both sides of the body, and may appear to have a central sore encircled by pale red rings (known as a bulls-eye). This rash may erupt as painful blisters. […] Ulcers and other lesions in mucous membranes like the mouth, eyes and genital regions.

• #16 Stevens-Johnson syndrome

  https://www.myamericannurse.com/stevens-johnson-syndrome/

  Stevens-Johnson syndrome is a life-threatening necrotic rash associated with various medications, including anticonvulsants. […] Ms. Davidson arrives in the emergency department (ED) with a painful rash that has progressed to blisters. The blisters appear on the skin and the mucus membranes of the mouth, throat, eyes, genitals, and anus. Ms. Davidson reports that cough and body aches preceded the rash. […] SJS may begin with flu-like symptoms, followed by a maculopapular rash that progresses to a pustular or bulbous rash. Rashes can produce skin sloughing, exposing underlying skin layers and causing extreme pain, hemorrhage, heat loss, and massive fluid and protein loss. […] If the rash is minor, treatment includes stopping the agent and administering diphenhydramine 25 to 50 mg twice a day. For more severe rashes, treatment includes prednisone 10 mg twice a day.

• #16 Stevens-Johnson syndrome

  https://www.myamericannurse.com/stevens-johnson-syndrome/

  Patients experiencing severe symptoms require hospital admission and laboratory studies to monitor for developing anemia, neutropenia, thrombocytopenia, elevated erythrocyte sedimentation rate, and elevated blood urea nitrogen. […] SJS usually resolves within a few days, but severe cases may take several months. Instruct patients who’ve been treated for SJS to call 911 or go to the ED if symptoms recur.

• #17 Stevens-Johnson Syndrome (SJS): Causes and Treatments

  https://www.webmd.com/skin-problems-and-treatments/stevens-johnson-syndrome

  Stevens-Johnson syndrome may feel like the flu at first. Then you’ll get a rash that blisters, and your skin will peel. It can also affect your eyes and the inside of your mouth. […] SJS usually starts with a fever and feeling like you have the flu. A few days later, other symptoms appear, including: Painful red or purple skin that looks burned and peels off, Blisters on your skin, mouth, nose, and genitals, Red, painful, watery eyes. […] The SJS rash is sometimes described as looking like a target: concentric circles that are lighter around the edges and darker in the center. […] SJS is dangerous. If you have these symptoms, go to the emergency room. […] SJS starts with a flu-like illness. Your eyes may burn, too. Then, in 1 to 3 days, a rash will appear on your skin and on your mucous membranes. With treatment, your skin will heal in 2 to 3 weeks. SJS can start up to 2 weeks after you’ve stopped taking the medication that triggered it.

• #18 Stevens-Johnson syndrome

  https://logicalimages.com/visualdx/diagnosis/?diagnosisId=52342&moduleId=103

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute, severe eruptions manifesting desquamative skin lesions, multifocal mucositis, and systemic symptoms. SJS has been defined as involvement of detached and detachable skin that is less than 10% of body surface area (BSA), while TEN involves greater than 30% BSA. SJS-TEN overlap occurs when 10%-30% BSA is involved. However, the newer Niigata criteria define TEN as involvement of 10% BSA or greater. Note that SJS can rapidly evolve into TEN, and both can have an unpredictable clinical course. […] Importantly, cutaneous pain or burning skin is a prominent early feature of SJS/TEN, and its presence could signify an ominous sign of impending necrolysis. […] Cutaneous lesions may begin as a more typical exanthematous eruption that evolves to dusky, irregular, ill-defined coalescing macules with purpuric or detached centers. Lesions typically appear first on the central trunk, palms, and soles and then spread to involve the face and proximal extremities. As the disease progresses, large areas of serous blistering and sloughing may occur. Skin is typically painful and tender.

• #18 Stevens-Johnson syndrome

  https://logicalimages.com/visualdx/diagnosis/?diagnosisId=52342&moduleId=103

  Oral mucosal sloughing and crusting are present in more than 90% of cases, and ocular involvement is present in more than 80%. Urogenital and, more rarely, respiratory and gastrointestinal (GI) mucosa may also be involved. […] SJS carries a 1%-5% mortality risk, may or may not have systemic symptoms, and involves the trunk and face with many isolated lesions. TEN carries a 25%-35% mortality risk and invariably has systemic symptoms, and the lesions on the trunk and face are largely coalesced. […] SJS/TEN runs an unpredictable clinical course. It is typically preceded by nonspecific prodromal symptoms followed by characteristic skin and mucosal lesions.

• #19 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Dermatologic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/dermatologic-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten

  Within 1 to 3 weeks after the start of the offending medication, patients develop a prodrome of malaise, fever, headache, cough, and keratoconjunctivitis. Macules, often in a target configuration, then appear suddenly, usually on the face, neck, and upper trunk. These macules simultaneously appear elsewhere on the body, coalesce into large flaccid bullae, and slough over a period of 1 to 3 days. Nails and eyebrows may be lost along with epithelium. The palms and soles may be involved. Skin, mucosal, and eye pain are common. In some cases, diffuse erythema is the first skin abnormality of TEN. […] In severe cases of TEN, large sheets of epithelium slide off the entire body at pressure points (Nikolsky sign), exposing weepy, painful, and erythematous skin. Painful oral crusts and erosions, keratoconjunctivitis, and genital problems (eg, urethritis, phimosis, vaginal synechiae) accompany skin sloughing in most cases. Bronchial epithelium may also slough, causing cough, dyspnea, pneumonia, pulmonary edema, and hypoxemia. Glomerulonephritis and hepatitis may develop.

• #19 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Dermatologic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/dermatologic-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten

  Diagnosis is often obvious from appearance of lesions and rapid progression of symptoms. Histologic examination of sloughed skin shows necrotic epithelium, a distinguishing feature. […] Severe toxic epidermal necrolysis is similar to extensive burns; patients are acutely ill, may be unable to eat or open their eyes, and suffer massive fluid and electrolyte losses. They are at high risk of infection, multiorgan failure, and death. Mortality can be as high as 25 to 35% in adults but tends to be lower in children and with early treatment. With early therapy, survival rates for TEN approach 90%.

• #20 Guidelines for the management of Stevens–Johnson syndrome/toxic epidermal necrolysis: An Indian perspective – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/guidelines-for-the-management-of-stevens-johnson-syndrome-toxic-epidermal-necrolysis-an-indian-perspective/

  Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare, potentially life-threatening, severe mucocutaneous adverse reactions characterized by extensive epidermal detachment, erosion of mucosae and severe constitutional symptoms. […] The initial symptoms or prodrome include fever, upper respiratory tract symptoms and conjunctivitis mimicking febrile illness of infective origin. This is followed by the detachment of mucous membranes (oropharyngeal, conjunctival, anogenital and nasal). Usually, more than two mucous membranes are involved. Cutaneous lesions, in the form of dusky erythematous macules/purpura and/or flat typical/atypical target lesions, erupt in association with pain and burning sensation. […] The lesions extend symmetrically, predominantly on the trunk and proximal limbs over a period of hours to 2-3 days. There is an appearance of flaccid blisters followed by sheet-like detachment of epidermis.

• #20 Guidelines for the management of Stevens–Johnson syndrome/toxic epidermal necrolysis: An Indian perspective – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/guidelines-for-the-management-of-stevens-johnson-syndrome-toxic-epidermal-necrolysis-an-indian-perspective/

  Systemic symptoms are almost always associated with SJS/TEN overlap and toxic epidermal necrolysis. Involvement of the mucosae can lead to impaired alimentation, painful micturition, photophobia, diarrhea and respiratory distress. […] Re-epithelialization begins in a few days after the cessation of disease activity and is usually complete in about 3 weeks, barring mucosae and pressure sites which take longer. […] Complications can cause both mortality and long-term morbidity in SJS/TEN. There is an increased risk of sepsis due to altered immune function. Dyspigmentation and scarring may develop. Ocular sequelae such as sicca syndrome, synechiae, scarring and blindness may develop. Hypopharyngeal stenosis combined with dysphagia, and esophageal strictures are long-term complications that are difficult to treat.

• #21 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Skin Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/skin-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten

  Stevens-Johnson syndrome and toxic epidermal necrolysis usually begin with fever, headache, cough, keratoconjunctivitis (inflammation of the conjunctiva and the cornea in the eyes), and body aches. If caused by a medication, these symptoms usually appear 1 to 3 weeks after the start of the medication. Then the skin changes begin, with a flat red or purple rash on the face, neck, and trunk, often spreading later to the rest of the body in an irregular pattern. The areas of rash enlarge and spread, often forming blisters in their center. The skin of the blisters is very loose and easy to rub off, often with just a gentle touch or pull, and the blisters peel off over a period of 1 to 3 days. The affected areas are painful, and the person feels very ill with chills and fever. In some people, eyebrows and nails fall out. The palms and soles may be affected.

• #21 Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Skin Disorders – Merck Manual Consumer Version

  https://www.merckmanuals.com/home/skin-disorders/hypersensitivity-and-reactive-skin-disorders/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten

  In both disorders, sores appear on the mucous membranes lining the mouth, throat, anus, genitals, and eyes. The damage to the lining of the mouth makes eating difficult, and closing the mouth may be painful, so the person may drool. The eyes may become very painful and swell and become so crusted that they seal shut. The corneas can become scarred. The opening through which urine passes (urethra) may also be affected, making urination difficult and painful. Sometimes the mucous membranes of the digestive and respiratory tracts are involved, resulting in diarrhea and cough, pneumonia, and difficulty breathing. […] The extensive skin loss in toxic epidermal necrolysis is similar to a severe burn and is equally life threatening. People are very ill and may be unable to eat or open their eyes. Huge amounts of fluids and salts can seep from the large, raw, damaged areas. People who have this disorder are very susceptible to organ failure. They are also at risk of infection at the sites of damaged, exposed tissues. Such infections are the most common cause of death in people with toxic epidermal necrolysis. […] In toxic epidermal necrolysis, the death rate can be as high as 25 to 35% in adults and can be even higher in older adults with very severe blistering. The death rate in children is lower. […] In Stevens-Johnson syndrome, the death rate is lower than it is in toxic epidermal necrolysis.

• #22 Stevens-Johnson Syndrome – EyeWiki

  https://eyewiki.org/Stevens-Johnson_Syndrome

  The acute phase of SJS typically lasts between 8-12 days, and is followed by re-epithelialization of the denuded areas of skin. This re-epithelialization process occurs over the span of 2-4 weeks. […] Bilateral conjunctival hyperemia with purulent discharge is the most common ocular finding at initial presentation and is seen in 78% of acute SJS cases. […] As the disease course progresses, inflammatory changes to the ocular surface may lead to the development of bulbar and conjunctival ulcerations with subsequent pseudomembrane formation, epithelial sloughing, anterior uveitis, panophthalmitis, corneal ulceration, and corneal perforation. […] Following the acute phase of SJS, patients remain at risk for developing additional ocular sequelae. This includes symblepharon formation, forniceal shortening, Meibomian gland injury, entropion, trichiasis, punctal occlusion, limbal stem cell deficiency, corneal conjunctivalization, corneal neovascularization, as well as keratinization of the eyelid margins and ocular surface.

• #22 Stevens-Johnson Syndrome – EyeWiki

  https://eyewiki.org/Stevens-Johnson_Syndrome

  The overall incidence of chronic ocular surface changes is 21-29% in pediatric cases and 27-59% in adult SJS survivors. […] Current hypotheses for the pathogenesis of the late ocular surface changes observed in SJS survivors include persistent inflammation of the ocular surface following the acute injury and longstanding surface irritation secondary to repeated ocular surface trauma from adnexal changes incurred in the acute phase.

• #23 Stevens-Johnson Syndrome (SJS): Symptoms and Treatments

  https://www.allaboutvision.com/conditions/related/stevens-johnson-syndrome/

  Eye problems may worsen or change during each stage of Stevens-Johnson syndrome. Some people may also have ongoing eye issues after SJS, which can include: Photophobia (light sensitivity), Dry eye, Trichiasis (ingrown eyelashes), Symblepharon (adhesion within the conjunctiva), Eye pain, Reduced visual acuity (often due to ongoing complications of the ocular surface), Corneal scarring, Corneal neovascularization (formation of new blood vessels in the cornea), Conjunctival scarring, Inverted eyelids, Blindness.

• #24 Stevens-Johnson syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) – Treatment : Emergency Care BC

  https://emergencycarebc.ca/clinical_resource/clinical-summary/stevens-johnson-syndrome-sjs-and-toxic-epidermal-necrolysis-ten-treatment/

  The acute phase of SJS/TEN lasts 8-12 days, with persistent fever, mucous membrane involvement and epidermal sloughing, leaving large, raw painful areas of denuded skin. […] The SCORTEN score can be used to prognosticate the severity of disease. […] The SCORTEN is calculated based on 7 independent and easily obtainable lab values and has been found to accurately predict mortality. […] Score of 0 or 1 with slow disease progression and limited skin involvement can be treated on non-specialized wards. […] Scores of 2 or higher with more skin involvement or skin detachment >30% of BSA should be treated in the ICU or burn unit and transported to a specialized tertiary centre accordingly.

• #25 Stevens-Johnson syndrome – Diagnosis & treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/stevens-johnson-syndrome/diagnosis-treatment/drc-20355942

  Treating Stevens-Johnson syndrome requires hospitalization, possibly in an intensive care unit or a burn unit. […] If the underlying cause of Stevens-Johnson syndrome can be eliminated and the skin reaction stopped, new skin may begin to grow within several days. In severe cases, full recovery may take several months.

• #26 Stevens-Johnson Syndrome: Causes, Diagnosis, and Treatment

  https://www.healthline.com/health/stevens-johnson-syndrome

  The rash usually starts on the face and chest before spreading to the mucous membranes, most often your lips and the inside of your mouth, but it could also appear on your: eyes and eyelids, genitals, esophagus, upper respiratory tract, anus. […] In some places, the rash will blister and the epidermis the outermost layer of your skin dies and peels. […] Symptoms might resemble a flu at first, but a rash will usually appear within a few days. The rash will spread to the mucous membranes and will peel and blister. […] Recovery from SJS will look different for everyone, depending on the severity of symptoms and complications. SJS is sometimes fatal reportedly 2 to 5 percent of cases but these numbers have been improving as treatments get better. […] Skin will start to regrow in 2 to 3 weeks but could take several months to fully return. Other symptoms or complications will have a range of recovery times and can have permanent effects.

• #27 Stevens–Johnson syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Stevens%E2%80%93Johnson_syndrome

  Stevens-Johnson syndrome (SJS) is a type of severe skin reaction. Early symptoms of SJS include fever and flu-like symptoms. A few days later, the skin begins to blister and peel, forming painful raw areas. Mucous membranes, such as the mouth, are also typically involved. Complications include dehydration, sepsis, pneumonia and multiple organ failure. […] SJS usually begins with fever, sore throat, and fatigue, which is commonly misdiagnosed and therefore treated with antibiotics. Patients with these disorders frequently experience burning pain of their skin at the start of disease. 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 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. […] Skin usually regrows over two to three weeks; however, complete recovery can take months. Overall, the risk of death with SJS is 5 to 10%.

• #28 Stevens-Johnson syndrome | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/stevens-johnson-syndrome?content_id=CON-20257415

  Stevens-Johnson syndrome (SJS) is a rare, serious disorder of the skin and mucous membranes. It’s usually a reaction to medication that starts with flu-like symptoms, followed by a painful rash that spreads and blisters. Then the top layer of affected skin dies, sheds and begins to heal after several days. […] One to three days before a rash develops, you may show early signs of Stevens-Johnson syndrome, including: Fever, A sore mouth and throat, Fatigue, Burning eyes. […] As the condition develops, other signs and symptoms include: Unexplained widespread skin pain, A red or purple rash that spreads, Blisters on your skin and the mucous membranes of the mouth, nose, eyes and genitals, Shedding of skin within days after blisters form. […] Stevens-Johnson syndrome requires immediate medical attention. Seek emergency medical care if you experience signs and symptoms of this condition. Drug-induced reactions may occur during the use of a medication or up to two weeks after discontinuing it. […] If the underlying cause of Stevens-Johnson syndrome can be eliminated and the skin reaction stopped, new skin may begin to grow within several days. In severe cases, full recovery may take several months.

• #29 Stevens-Johnson syndrome/toxic epidermal necrolysis: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/stevens-johnson-syndrome-toxic-epidermal-necrolysis/

  Severe damage to the skin and mucous membranes makes SJS/TEN a life-threatening disease. Because the skin normally acts as a protective barrier, extensive skin damage can lead to a dangerous loss of fluids and allow infections to develop. Serious complications can include pneumonia, overwhelming bacterial infections (sepsis), shock, multiple organ failure, and death. About 10 percent of people with Stevens-Johnson syndrome die from the disease, while the condition is fatal in up to 50 percent of those with toxic epidermal necrolysis. […] Among people who survive, long-term effects of SJS/TEN can include changes in skin coloring (pigmentation), dryness of the skin and mucous membranes (xerosis), excess sweating (hyperhidrosis), hair loss (alopecia), and abnormal growth or loss of the fingernails and toenails. Other long-term problems can include impaired taste, difficulty urinating, and genital abnormalities. A small percentage of affected individuals develop chronic dryness or inflammation of the eyes, which can lead to increased sensitivity to light (photophobia) and vision impairment.

• #30

  https://step2.medbullets.com/dermatology/120088/stevens-johnson-syndrome

  A young boy is brought to the emergency room after visiting his primary care physician, who noted erythematous, desquamative lesions all over his body (well over 30% of body surface area) and ulcerations of the mucosal membranes of the mouth and eyes. He was recently treated with penicillin for an infection. […] Symptoms […] very painful skin […] systemic signs […] fever […] dehydration […] hypotension […] non-skin findings […] ocular involvement (50-80%) […] corneal ulceration […] uveitis […] pulmonary involvement (25%) […] pneumonitis […] bronchiolitis obliterans […] bronchitis […] Physical exam […] initially dusky red macules or patches (not raised) that progress to tense bullae and eventual skin sloughing […] mucous membranes always involved […] bullae and erosions in oral, genital, anal mucosa […] Most common cause of death is sepsis […] High mortality […] TEN: 30 – 50% mortality rate […] SJS: 5 – 10% mortality rate.

• #31 Stevens-Johnson Syndrome – MD Searchlight

  https://mdsearchlight.com/skin-problems-and-treatments/stevens-johnson-syndrome/

  In another study conducted in Crteil, France, out of 361 patients diagnosed with either SJS or TEN, 66 people unfortunately passed away. That’s a mortality rate of 18%: 2% among those with SJS, 12% among those with overlapping SJS/TEN, and a notably higher 26% among those with TEN. […] In the early stages, the biggest risk of Stevens-Johnson Syndrome (a severe skin reaction) is sepsis, a potentially life-threatening condition caused by the body’s response to an infection. Other risks include failure of the lungs, liver, and kidneys. […] The long-term complications of Stevens-Johnson Syndrome can affect the eyes (even leading to blindness), skin (causing changes in color and scarring), and kidneys. The skin and mucous membranes can form blisters and sores which may result in strictures (abnormal narrowing) and scars.

• #32 Stevens Johnson syndrome – Overview of Information and Clinical Research

  https://clinicaltrials.eu/disease/stevens-johnson-syndrome/

  The prognosis for individuals with SJS varies depending on several factors, including the severity of the condition and the timeliness of treatment. The mortality rate for SJS is approximately 7.5%, with a higher risk associated with more extensive body surface involvement. In cases where the condition progresses to toxic epidermal necrolysis (TEN), the mortality rate can increase significantly, reaching up to 30-40%. Early intervention and management are crucial in improving outcomes and reducing mortality rates. […] Living with SJS involves adapting to the challenges posed by the condition and its potential long-term effects. Recovery can take weeks to months, and patients may experience fatigue and other symptoms during this period. It is important for patients to avoid the medication that triggered the initial reaction, as re-exposure can lead to more severe episodes.

• #33 Stevens-Johnson syndrome: Symptoms, causes, and treatment

  https://www.medicalnewstoday.com/articles/326587

  Stevens-Johnson syndrome is a severe skin condition that can develop in response to certain medications or infections. People with Stevens-Johnson syndrome require immediate medical attention. […] Despite the many treatment options, people can die from Stevens-Johnson syndrome. Individuals with more severe forms of Stevens-Johnson syndrome have higher mortality rates. Older adults and people with other underlying medical conditions also have a higher risk of dying.

• #34 Toxic Epidermal Necrolysis in Children

  https://www.nationwidechildrens.org/conditions/health-library/toxic-epidermal-necrolysis-in-children

  Symptoms can be a bit different for each child. They can include: […] A high fever and flu-like symptoms often occur first. This is followed by skin changes such as painful redness, peeling, blistering, and raw areas of skin. […] The disease progresses fast, often within 3 days. […] Toxic epidermal necrolysis can be life-threatening. If your child has these symptoms, take them to the closest emergency room for assessment.

• #35 A to Z: Stevens-Johnson Syndrome (for Parents) – Aetna Better Health of Kentucky (Medicaid)

  https://kidshealth.org/AetnaBetterHealthKentucky/en/parents/az-sjs.html

  Stevens-Johnson syndrome is a rare inflammatory disorder affecting the skin and mucous membranes. It’s usually triggered by exposure to an infection or a medication. […] In Stevens-Johnson syndrome, the immune system overreacts to a medication or infection. This causes flu-like symptoms, fever, blistering of the mucous membranes, and a red or purplish rash. In places, the top layer of skin may separate from the underlying layers, blister, and shed, leaving raw, exposed skin. […] SJS usually affects the mouth, nose, and eyes, but also can occur in the mucous membranes lining the ears; eyes; vagina; and urinary, respiratory, and gastrointestinal tracts. […] Stevens-Johnson syndrome is a serious condition that requires immediate medical attention. If the trigger of the condition can be identified and avoided, a recurrence of Stevens-Johnson syndrome is unlikely. However, some cases happen without any known trigger. […] In a similar but more severe form of the condition known as toxic epidermal necrolysis, greater areas of skin shedding occurs that can be life threatening.

• #36 Effectiveness of early treatment with plasma exchange in patients with Stevens–Johnson syndrome and toxic epidermal necrolysis | Scientific Reports

  https://www.nature.com/articles/s41598-024-53653-5

  Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) are potentially fatal medical conditions that lack established treatment. […] The mainstay of management is supportive treatment, which includes discontinuation of causative agents, fluid replacement therapy, local treatment, nutritional support, and respiratory management. […] Therapeutic plasma exchange (PE) is another treatment option but evidence of its effectiveness is limited to case reports and small case series. […] The rationale for PE in SJS/TEN is to filter causative drugs and their metabolites, and remove immunological factors involved in the disease. […] The effectiveness of initiating PE immediately on hospital admission in patients with severe SJS/TEN was evaluated using a nationwide database and adjusted using a doubly robust estimation method to minimise confounding. The results showed no benefit of PE for reducing in-hospital mortality or the length of hospital stay. […] This study, with appropriate adjustments for concurrent treatment, provided no evidence that PE reduced mortality or shortened the length of hospital stay. […] In contrast to previous studies, this study did not find any evidence of a benefit of early PE in patients with severe SJS/TEN.

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