steven johnson syndrome

Editor's Notes

1. Q DDx , Tx Answer 37: C The patient’s constellation of symptoms, a diffuse nonblanching rash, conjunctivitis, and mucositis, is concerning for Stevens-Johnson Syndrome(SJS), which is an immune-mediated reaction. The customary physical findings described result due to the dermis separating from the epidermis, causing extensive skin necrosis, and leading to increased risk for infection, as well as potential respiratory compromise secondary to local edema and sloughing of the airway tissue. As such, the best step in managing this patient is to optimize hydration and admit to the intensive care for close monitoring and aggressive airway protection if the patient’s condition worsens. While his rash could occur due to a systemic bacterial infection, starting antibiotics and admitting him to a general ward without addressing his risk for airway compromise is the incorrect choice. Similarly, treating this patent for impetigo as an outpatient is also incorrect. Lastly, while a community-acquired pneumonia caused by Mycoplasma pneumoniae has been identified as a potential cause of SJS, simply treating the patient with antibiotics as an outpatient would be incorrect. Erythema multiforme is not associated with severe skin necrosis and is often referred to as EM minor when the rash is limited to the skin (Figure 3), and EM major when the mucosa becomes involved. Stevens-Johnson syndrome denotes patients with skin necrosis that involves between 10% to 30% of the body surface, while TEN typically is reserved for more severe cases when greater than 30% of body surface is involved. Patients with even limited skin findings that suggest these conditions require close observation to monitor for progression of disease. In addition, rashes that persist for extended periods and are associated with systemic symptoms should also prompt further evaluation for rheumatologic disorders like systemic lupus erythematosus and dermatomyositis or inflammatory conditions like mucocutaneous lymph node syndrome (Kawasaki disease). There are several physical examination findings that can also help differentiate a serious rash. This first is whether it “blanches” or not, meaning whether the rash’s color fades when pressure is applied and then return when the pressure is removed. While rashes that blanch are not uniformly innocent, they primarily result from vasodilation due to local inflammation or irritation and usually are part of a self-limited illness. Rashes that do not blanch, however, should trigger further investigation as they result from extravasation of blood into the skin or soft tissue. These red or discolored spots are traditionally referred to petechial if are less than 3mm, purpura when they are between 3 to 10 mm, and ecchymosis when they are larger than 10 mm. These lesions can occur from local trauma or pressure, however, they can also result from more serious conditions. These include infection, such as Rocky Mountain Spotted Fever (Figure 4), vasculitis such as Henoch–Schönlein purpura (Figure 5), or bleeding disorders such as thrombocytopenia from idiopathic thrombocytopenic purpura or disseminated intravascular coagulation from a systemic infection. The other physical finding that can separate life threatening rashes from less serious ones is if the patient’s skin sloughs after gentle lateral pressure, also referred to as a positive Nikolsky sign. This occurs when the dermis separates from the epidermis due to a serve immune mediated reaction and is associated with several conditions, including SJS (like the patient is our vignette), TEN, staphylococcal scalded skin, and pemphigus vulgaris. Patients with these conditions are at risk for severe fluid loss, airway compromise, and sepsis, and should be cared for at a facility with experience in managing extensive skin loss such as a burn center.
2. Include adult and pediatrics , Very old statiscs
3. More necrosis in TEN cutaneous and mucosal lesions (EM major) cutaneous lesions alone (EM minor)
4. Few cases reported . A weak association with acetaminophen/paracetamol has also been reported [20] but remains doubtful, as these drugs are frequently given to treat the prodromal or early symptoms of the disease (fever, headache, malaise, burning eyes, burning mouth) [22]. Furthermore, a history of previous tolerated use of these drugs makes their association with SJS/TEN even more unlikely. * Significant association in case-control studies with a lower limit of the confidence interval ≥5 and unpublished data from the RegiSCAR. association in case-control studies with a lower limit of the confidence interval <5.
5. Other infection is CMV , hepatitis A , herpes Other predisposing conditions include HIV (100-fold higher risk), malignancy, systemic lupus erythematosus, radiotherapy, collagen vascular disease, UV light, genetics, and underlying immunologic disease.
6. "target lesion". begin with ill-defined erythematous macules with purpuric centers, diffuse erythema The skin is often tender to the touch, and skin pain out of proportion to the cutaneous findings. start on the face and thorax before spreading to other areas and are symmetrically distributed . Atypical target lesions with darker centers may be present.
7. Mucosal Lesion at mouth, eyes, genitals occurs in 90 percent of and can precede or follow the skin eruption Painful crusts and erosions may occur on any mucosal surface Oral mucosa and the vermilion border are almost involved, with painful hemorrhagic erosions and crusts covered with a grayish-white membrane . Stomatitis and mucositis lead to impaired oral intake with consequent malnutrition and dehydration. Ocular involvement in approximately 80 percent of patients. The most common change in the eyes is a severe conjunctivitis with a purulent discharge , but bullae may develop. Corneal ulceration is frequent, and anterior uveitis or panophthalmitis may occur. Pain and photophobia are accompanying symptoms. severe conjunctivitis with purulent discharge, swollen and erythematous eyelids, suppurative keratitis, endophthalmitis, pain, and photophobia Urogenital — Urethritis develops in up to two-thirds of patients and may lead to urinary retention. Genital erosions are frequent. vulvovaginitis, and urologic (urethritis).
8. As the disease progresses, vesicles and bullae form, and within days the skin begins to slough. Nikolsky sign may be positive Epidermal detachment with Nikolsky's sign of the bullae (sloughing of the superficial skin layer with slight rubbing pressure) then presents 3 to 5 days later. Other — Pharyngeal mucosa is affected in nearly all patients; tracheal, bronchial, and esophageal membranes are less frequently involved . Intestinal involvement is rare (pneumonia, interstitial pneumonitis, acute respiratory distress syndrome, mechanical ventilation in 25% 39 with its as- sociated higher mortality 40 gastrointestinal (diarrhea, melena,small bowel ulcerations, colonic perforation, small bowel intussusception, stenosis, strictures, stomatitis)
9. due to massive transdermal fluid loss and hypercatabolic state
10. A skin biopsy for routine histopathologic examination and possibly direct immunofluorescence is useful in confirming the diagnosis and excluding other conditions that may mimic SJS/TEN. LABS , MYCOPLASMA PNEUMONIA PCR bulla spread sign When press on vesicle or bulla , it is regular and round border
11. systemic involvement of SJS and TEN compared with the epidermal necrosis of SJS and TEN heavily exudative conjunctivitis in SJS compared with SJS and TEN onset within 8 weeks of drug initiation DHS/DRESS (drug hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms) : Maculopapular exanthem , Generalized Erythroderma , Facial edema , Mucosal involvement infrequent Lymphadenopathy Hepatitis Pneumonitis Eosinophilia, atypical lymphocytes
12. Proven to be effective
13. Silver sulfadiazine should be avoided if SJS/TEN is suspected to be caused by sulfonamides, but silver nitrate and silver-imbued nanocrystalline gauze materials may be used safely
14. pain control : wound care is cause more pain Sterile handling is essential Antiseptic solutions containing octenidine, polyhexanide (eg, Octenisept, Lavasept, Prontosan), or chlorhexidine  or silver nitrate preparations may be used for disinfection. Repeated cultures of the skin, as well as blood, catheters, gastric, and urinary tubes, should be obtained at 48-hour intervals
15. no RCT studies in pediatrics , but in adult there is very few randomized prospective trials due to rarity of the condition and ethical considerations. the most used promising being IVIG, and corticosteroids. , best study done Over all, studies for IVIG and corticosteroids have shown minimal standardization for medication dosing, treatment duration, and outcome measures, among others.
16. Scoreten up to 7 The pediatric-modified SCORETEN were not superior to the adult SCORTEN, which accurately predicted outcome Morbidity in children with SJS or TEN can be predicted through the use of severity of illness scores, including SCORTEN, ABSI, PIM2, and PRISM III
17. With initial nonspecific manifestations and high mortality rates
18. Typical is like erythema multiform