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Stevens Johnson Syndrome And Toxic Epidermal Necrolysis Pdf

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Supportive care remains the mainstay of treatment, and includes wound care, fluid and electrolyte management, management of medical co-morbidities, and infection control. The value of adjuvant therapy remains unclear, but new recent retrospective studies suggest that the combination therapies may be efficacious. Supportive care is the most universally accepted therapy, although specific strategies may vary among institutions. Adjuvant therapies include corticosteroids, IVIG, cyclosporine, TNF alpha inhibitors, and plasmapheresis but prospective data is still lacking.

Management of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis: a Review and Update

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. Based on the similar histologic findings, SJS and TEN were synonymously associated with erythema multiforme major since However, Bastuji-Garin et al. The present understanding of epidermal necrolysis is that it is an immune-driven pathway mediated by granulysin released by drug-specific cytotoxic CD8 T cells and natural killer cells.

This is followed by the detachment of mucous membranes oropharyngeal, conjunctival, anogenital and nasal. Usually, more than two mucous membranes are involved. Typical raised target lesions, characteristic of the erythema multiforme spectrum, are usually absent. 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. Shearing pressure on the involved erythematous skin may cause epidermal detachment pseudo-Nikolsky's sign. Peri-lesional erythema is a sign of disease activity and helps to monitor response to treatment. Involvement of the mucosae can lead to impaired alimentation, painful micturition, photophobia, diarrhea and respiratory distress. Thermoregulation is impaired and energy expenditure is increased.

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. 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. Synechiae in other mucosae such as mouth and genitalia esophagus or vaginal stenosis may require surgery.

The management essentials include early recognition of the condition, cessation of suspected drug s if any, prompt institution of supportive therapy, referral if required, initiation of specific therapy, management of complications and prevention of future episodes. The ideal therapy still remains a matter of debate as there are only a limited number of studies of good quality comparing the usefulness of different specific treatments.

The management and overall prognosis depend on the stage at which treatment is initiated, age of the patient, extent of necrolysis, associated comorbidities, accompanying complications electrolyte imbalance, renal or hepatic dysfunction, adult respiratory distress syndrome and sepsis , the patient's ability to pay, drugs and resources available for patient care and the physician's experience with their use. Prognostication is a complex exercise where the clinician has to take into account several parameters.

Outcomes are based on several clinical and lab oratory parameters, age of the patient, co-morbidities, co-existing compromised disease states and physiologic states such as pregnancy. It is useful to predict the mortality and severity of illness.

The index identifies seven independent risk factors for death [Box 1]. Each parameter is given a score of one and the total score is calculated by summing up the number of abnormal parameters. The most common causes of death are sepsis, pulmonary failure and multiple organ failure. This consensus statement would be valid for 3 years from the date of publication when the document will be reviewed for validity based on the current best evidence at that time. Based on the inputs received, the final consensus statement was prepared.

A total of articles meta-analyses, prospective and retrospective studies, reviews [including chapters in books], previous guidelines [including Indian guidelines of and UK guidelines of ] and case series were critically evaluated and the evidence thus gathered was used in the preparation of these guidelines. Withdrawal of the offending drug will achieve the objective of aborting or slowing the process of acute skin failure and allow skin epithelialization in a shorter period of time.

It has been shown that the earlier the causative drug is withdrawn, the better the prognosis and that patients exposed to causative drugs with long half-lives have an increased risk of dying.

When the culprit drug is identified, it is easier to withdraw the offending drug. However, when multiple drugs polypharmacy or multi-drug combination therapies are used, it is difficult to ascertain the exact drug responsible for causing a drug reaction.

However, if administration of a drug is absolutely essential especially relevant for epileptics and also severe life-threatening infections such as septicemia , these drug s can be substituted with structurally unrelated drug s.

It is important to restrict drug intake to the minimum possible. A detailed history must encompass the list of all drugs including herbal, indigenous and other alternative system of medications taken within a period of 2 months prior to the onset of the eruption.

Co-morbidities infections, immunosuppression, hepatic and renal disease, connective tissue disorders and chronic diseases such as diabetes mellitus and hypertension should also be noted. The temporal association with the drug intake and previous episodes of allergic reactions are important parameters to incriminate a drug as an etiological agent.

Personal or family history of drug reactions may be contributory and this must be recorded. An initial assessment should include evaluation of airway, breathing and circulation, urinary output and any clinical evidence of septicemia.

Percentage of body surface area involved and degree and the extent and number of mucosae involved need to be assessed and documented. The extent of erythema and the epidermal detachment, both detachable epidermis i. Nikolsky-positive , as well as detached epidermis, should be recorded and depicted on a body map in the medical records.

The same may also be documented with the help of photographs taken at the time of admission and repeated during the treatment at various stages. This helps to serve as an important safeguard against any potential medicolegal litigation. Unfortunately, a confirmatory laboratory test that will allow the diagnosis of a drug reaction with some degree of certainty and identify the culprit drug is presently not available and those mentioned in the current literature are in experimental stages and have been used for research purposes.

Hence, the recommended investigations for all the patients are for the purpose of knowing the degree of underlying damage to various organ systems, for prognosis and to plan further management [Appendix 1]. It should be noted that the severity of systemic damage may not necessarily correlate with that of epidermal necrolysis. The investigations include the following:.

Hospitalization in an intensive care setting or burns unit may be considered where such facilities are available. If possible, the patient should be admitted to an isolation room to facilitate infection and temperature control. Owing to the multisystem nature of disease, management should preferably be carried out by a team of experts including a dermatologist, physician, ophthalmologist, plastic surgeon, chest physician, dietician and any other specialists required as needed.

Supportive treatment is essentially the same as for burn patients. Frequent monitoring of vital signs is an essential part of management as they offer the first sign of a worsening systemic condition. Monitoring both initially at baseline and subsequently at periodic intervals includes parameters such as pulse rate, blood pressure, respiratory rate, fluid intake and urine output chart, blood glucose, serum electrolytes, serum creatinine and specific cultures.

When the patient is on immunosuppressives, one must monitor for foci of sepsis dental, gastrointestinal and urinary that may flare up during the course of disease.

Complications such as septicemia and disseminated intravascular coagulation can be monitored by specialized tests such as coagulation assays, D-dimer assay and fibrin degradation products, where facilities are available. In case barrier nursing is available, blood cultures are done at admission and then every 48 h. It is preferable to obtain the swab for culture and sensitivity from three lesional sites, particularly sloughed or crusted areas on alternate days throughout the acute phase. It should be kept in mind that systemic steroids may mask the signs of sepsis.

Since most centers do not have the infrastructure for barrier nursing, prophylactic antibiotic therapy may be considered for widespread skin involvement and slightest clinical suspicion of sepsis.

After admission, an oral liquid diet, nasogastric tube or total parenteral nutrition should be initiated. If feasible, oral feeding is always preferred though it is often difficult because of upper gastrointestinal tract injury. Early and continuous enteral nutrition decreases the risk of stress ulcers, reduces bacterial translocation and enterogenic infection and allows early discontinuation of venous lines.

Proteins approximately 1. Regular cleaning of wounds with clean water or normal saline is important, whether the patient is bedridden or mobile, to remove the infected crusts as this reduces the chances of infection. Gentian violet paint in dilution and silver nitrate 0. For denuded areas, dressing can be done with paraffin or petrolatum gauze, with or without antibiotic impregnation.

Adhesive dressings should be avoided. Burn cage is also useful in preventing adhesion of denuded areas to clothes in cases of extensive involvement. Oral hygiene should be maintained with normal saline swishes or antiseptic or anesthetic mouth washes. Saline compresses followed by the application of lubricants can be advised for the lips.

This also helps soften hemorrhagic lip crusts. Daily examination by an ophthalmologist and vigorous treatment reduce the risk of long-term ocular complications. Lid globe adhesions should be cautiously removed with a glass rod daily. Recently, the application of amniotic membranes was reported to be effective in preserving visual acuity and an intact ocular surface.

Lung involvement may be complicated by pulmonary edema during fluid replacement. Pulmonary care includes normal saline aerosols, bronchial aspiration and postural drainage by turning the patient to different sides. Pooling of saliva and secretions may predispose to aspiration and therefore need to be cleared frequently. Hypostatic pneumonia should be prevented by frequent change of posture and mobilization of the patient as early as possible.

The nose may require attention in the form of moisturization with saline and removal of adherent crusts. Antacids H 2 receptor antagonists and proton pump inhibitors reduce the incidence of gastric bleeding. Pain management has to be individualized taking into consideration the clinical needs of the patient, viable route of administration, risk of respiratory suppression and monitoring facilities. Opioids such as tramadol and anxiolytics may be helpful. Thromboembolism and disseminated intravascular coagulation are important causes of morbidity and death and hence, anticoagulation with low-molecular weight heparin may be useful in some patients.

The laboratory changes commonly include anemia and lymphopenia; neutropenia is seen in one-third of the patients and is associated with a poor prognosis. Hyperglycemia and glycosuria may occur due to stress, infection and possibly pancreatitis and indicate a poor prognosis. Liver enzymes are elevated in half of the patients and occasionally, frank hepatitis may develop, induced by drugs, sepsis or shock. Hematuria and proteinuria may be seen indicating renal involvement.

Blood urea nitrogen and serum creatinine levels may be raised because of dehydration. Serum electrolyte levels may guide fluid and electrolyte administration. Chest radiograph, blood and skin swab culture, human immunodeficiency virus testing enzyme-linked immunosorbent assay and skin biopsy may be done if indicated.

Traditionally, systemic corticosteroids have remained the mainstay of therapy of Stevens-Johnson syndrome and toxic epidermal necrolysis in most centers.

The largest study to evaluate the effect of treatments, including steroids, was performed by Schneck et al. The authors concluded that there was inadequate evidence that any specific treatment is established as effective with only corticosteroids showing a trend for possible benefit. Among Indian studies, Pasricha et al. Ocular sequelae are one of the major complications of Stevens-Johnson syndrome and toxic epidermal necrolysis.

2020, Number 01

Clinical patterns and classification of the diseases included in the study. A, Erythema multiforme: typical targets, with regular round shape, well-defined borders, 3 different zones, predominant on the extremities. B, Stevens-Johnson syndrome: erythematous or purpuric macules with irregular shape and size. Blisters often occur on all or part of the macule. Lesions are widespread. All photographs were printed from digital records of the original color photographs with no other change than magnification.

If your institution subscribes to this resource, and you don't have a MyAccess Profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus. Please consult the latest official manual style if you have any questions regarding the format accuracy. Toxic epidermal necrolysis TEN and Stevens—Johnson syndrome SJS are acute life-threatening mucocutaneous reactions characterized by extensive necrosis and detachment of the epidermis. Stevens and Johnson first reported two cases of disseminated cutaneous eruptions associated with an erosive stomatitis and severe ocular involvement. Because of the similarities in clinical and histopathologic findings, risk factors, drug causality, and mechanisms, these two conditions are now considered severity variants of an identical process that differs only in the final extent of body surface involved. Epidermal necrolysis EN is rare. Patients infected with human immunodeficiency virus and to a lesser degree patients with collagen vascular disease and cancer are at increased risk.

Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis

If your institution subscribes to this resource, and you don't have a MyAccess Profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus. Please consult the latest official manual style if you have any questions regarding the format accuracy. Widespread apoptosis of keratinocytes is provoked by the activation of a cell-mediated cytotoxic reaction and amplified by cytokines, mainly granulysin. Confluent purpuric and erythematous macules evolving to flaccid blisters and epidermal detachment often start on the upper trunk and spread to the limbs associated with mucous membrane involvement. Histopathology shows full-thickness necrosis of epidermis associated with mild mononuclear cell infiltrate.

Erythema multiforme in its most extreme form has traditionally been divided between toxic epidermal necrolysis and Stevens-Johnson Syndrome. These two life-threatening skin diseases are now considered part of the same spectrum of disease. They can be differentiated by clinical and histological criteria. We can also now predict which patients are apt to have the most guarded prognosis. Treatment by multiple agents is imperfect, but offers a better chance of a good outcome than ever before.

Stevens-Johnson syndrome SJS and toxic epidermal necrolysis TEN , which can be considered a late-onset allergic reaction, can cause serious long-term sequelae.

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3 Comments

Diathikomra1971 19.03.2021 at 10:12

If your institution subscribes to this resource, and you don't have a MyAccess Profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Viviano M. 19.03.2021 at 15:45

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.

Gracie R. 20.03.2021 at 01:54

Stevens-Johnson syndrome SJS and toxic epidermal necrolysis TEN are two of the most severe dermatologic conditions occurring in the inpatient setting.

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