DermKnowledgeBASE: Stevens johnson Syndrome

Stevens johnson Syndrome

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The text is the summary of recent articles on Stevens johnson Syndrome at 75 thresold from National Library of Medicine (NLM). This information is subject to NCBI's Disclaimer and Copyright notice.

In opposite to the main cutaneous allergic drug reactions such as urticaria or Maculopapular skin rash, in which Antibiotics are the main culprits, in severe drug allergic reactions such as SJS (Stevens-Johnson Syndrome), TEN (Toxic Epidermal Necrolysis), or DRESS (Drug Reaction with Eosinophilia and Systemic Symptom Syndrome) compounds like allopurinol and anticonvulsants are the main causes [1]. In the past 30 years, tremendous progress has been made in understanding the causes and pathobiology of this often life-threatening condition [2]. Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe reactions affecting skin and Mucosa with blisters and Erosions [3]. Adverse cutaneous manifestations in response to imat-inib are not infrequent and can include dry skin, Alopecia, facial Edema, and photosensitivity rash [4]. Other less common manifestations include exfoliative dermatitis, nail disorders, psoriasis, folliculitis, hypotrichosis, urticaria, petechiae, Stevens-Johnson syndrome, erythema multiforme, Sweet syndrome, and leukocytoclastic Vasculitis [5].

(7)Division of Allergy Immunology and Rheumatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand [6]. Regarding extra-cutaneous manifestations, hepatomegaly was observed in two cSLE patients, nephritis in two and neuropsychiatric involvement and conjunctivitis were observed respectively in one patient [7]. All patients were treated with Steroids and four needed additional treatment such as intravenous immunoglobulin in two patients, hydroxychloroquine and Azathioprine in two and intravenous Cyclophosphamide in one patient [8]. Interest in cyclosporine, a calcineurin inhibitor that can block the function of T-cells, has increased with the discovery of the importance of granulysin in apoptosis in toxic Epidermal necrolysis [9]. Five patients in the supportive only group and one in the cyclosporine group died [10].

The second DHR includes exanthema, erythroderma, DRESS, Stevens-Johnson syndrome/toxic Epidermal necrolysis (SJS/TEN), hepatitis, and agranulocytosis [11]. Despite the well-described ocular and orofacial manifestations of SJS/TEN, there is a paucity of reports on the genitourinary (GU) symptoms and their management [12]. Incidences of GU manifestations at presentation and their management are shown in Summary Table [13]. (4)Department of Dermatology, University of Pennsylvania, Philadelphia [14]. (9)Department of Molecular and Clinical Pharmacology, University of Liverpool, Liverpool, England [15].

(14)Allergy Immunology and Rheumatology Division, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand [16]. (2)Chang Gung Immunology Consortium, Chang Gung Memorial Hospital and Chang Gung University, Taoyuan, Taiwan [17]. The purpose of this report was to present two cases with significant variations in the clinical presentation of Stevens-Johnson syndrome [18]. We present the case of a 14-year-old Female patient with acute pancreatitis as an initial manifestation of systemic lupus erythematosus, complicated by the toxic Epidermal necrolysis with a fatal outcome [19]. Systemic lupus erythematosus was diagnosed secondary when her condition has been already complicated by the toxic Epidermal necrolysis [20].

There were 10 cases for each symptom, namely erythema multiforme and Stevens-Johnson Syndrome, observed in this study [21]. The most concerning of these adverse reactions is Stevens-Johnson syndrome/toxic Epidermal necrolysis [22]. We performed a systematic review of randomized controlled trials using lamotrigine as a monotherapy to quantify the incidence of cutaneous reactions, particularly Stevens-Johnson syndrome/toxic Epidermal necrolysis [23]. (2)Associate Professor, Department of Pharmacology, Kasturba Medical College, Manipal University , Manipal, Karnataka, India [24]. The co-existence of Stevens-Johnson syndrome and agranulocytosis in the same patient further increases the risk of morbidity and mortality [25].

To the best of our knowledge, there are no reports available in the existing literature, of cases that were reported with both these life-threatening conditions in a single patient, at the same point of time [26]. They have some commonalities such as nonimmediate nature and T-cell mediation and rare overlap syndromes have been documented, most commonly involving acute Generalized Exanthematous pustulosis (AGEP) and drug rash with eosinophilia and Systemic symptoms (DRESS), and DRESS and toxic epidermal necrolysis (TEN) [27]. She received intravenous Cyclosporine during the admission that resulted in acute Kidney injury and the therapy was discontinued [28]. The Skin Biopsy ruled out Stevens-Johnson syndrome and was more consistent with generalized pustular psoriasis [29]. She was diagnosed with acute Generalized and erythrodermic psoriasis and the patient was restarted on Cyclosporine 100 mg twice a day [30].

To our knowledge, acute generalized erythrodermic pustular psoriasis associated with bupropion/naltrexone has not been reported in a patient without history of psoriasis [31].

References: 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 , 18 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30 , 31 ,

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Bell Raj Eapen
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