scholarly journals MANAGEMENT OF ORAL LESIONS ASSOCIATED WITH CARBAMAZEPINE RELATED STEVENS-JOHNSON SYNDROME / TOXIC EPIDERMAL NECROLYSIS OVERLAP PATIENT

2016 ◽  
Vol 19 (2) ◽  
pp. 154-159
Author(s):  
Dewi Puspasari ◽  
Irna Sufiawati
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
Fas Ligand ◽  
Total Body Surface Area ◽  
Stevens Johnson Syndrome ◽  
Cytotoxic T Cell ◽  
Mean Corpuscular Hemoglobin ◽  
Oral Lesions ◽  
Treatment Standard ◽  
Johnson Syndrome ◽  
Mucosal Involvement

Stevens–Johnson syndrome (SJS)/ Toxic Epidermal Necrolysis (TEN) are acute, self-limited, potentially life-threatening mucocutaneous disease. Oral mucosal involvement manifest as extensive erosions and haemorrhagic crusting, which can interfere oral functions causing odynophagia, inability to tolerate solid foods, and increased aspiration risk. A 40-year-old female patient was referred from Dermatology and Venereology department with diagnosis SJS/TEN overlap. The patient complained mouth opening difficulty due to mouth and lip sores. Drug history revealed positive intake of carbamazepine. Extraoral examination revealed multiple diffuse discrete facial lesions, conjunctival hyperemia, erosions and hemorrhagic crusting lips. Intraoral examination revealed white yellowish plaque, and erosions on buccal mucosa, palate, floor of the mouth, dorsal, ventral, and lateral tongue. Laboratory investigation revealed decrease of haemoglobin, hematocrite, Mean Corpuscular Volume (MCV), Mean Corpuscular Hemoglobin (MCH), thrombocyte, eosinophil, band of eosinophil, lymphocyte, natrium, potassium, and calcium. Oral lesions associated with SJS/TEN overlap diagnosis was made. Chlorhexidine gluconate 0,1%, nystatin oral suspension, vitamin B12, folic acid, and corticosteroid unguent compounding were given, which showed improvement of oral lesions in 3 weeks. SJS/TEN are the same disease spectrum of delayed hypersensitivity reaction leading to keratinocyte apoptosis through cytotoxic T-cell mediated Fas-Fas ligand, perforin/ granzyme B, and granulysin, which distinguished primarily by severity and percentage of total body surface area involved.Currently, an optimal treatment standard for SJS/TEN patients remains unavailable. Oral lesions management play significant role in enhancing patients’ quality of life and achieving better prognosis in SJS/TEN overlap patients through multidisciplinary approach.

Head and Neck Manifestations of Erythema Multiforme in Children

1994 ◽  
Vol 111 (3P1) ◽  
pp. 236-242 ◽  
Author(s):  
Michael G. Stewart ◽  
Newton O. Duncan ◽  
Daniel J. Franklin ◽  
Ellen M. Friedman ◽  
Marcelle Sulek
Keyword(s):  
Oral Cavity ◽  
Toxic Epidermal Necrolysis ◽  
Head And Neck ◽  
Erythema Multiforme ◽  
Medication Use ◽  
Stevens Johnson Syndrome ◽  
Striking Increase ◽  
Johnson Syndrome ◽  
Mucosal Involvement ◽  
Mucous Membranes

Erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis are related disorders of skin and mucous membranes, which are typically associated with antecedent medication use or infection. We review 108 cases of erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis from Texas Children's Hospital, Houston, Texas, from 1981 to 1991, and illustrate the characteristic skin and mucosal lesions. In addition, we describe in detail two unusual cases requiring intensive airway management. Head and neck manifestations were present in 4 of 79 patients (5%) with erythema multiforme and 26 of 28 patients (93%) with Stevens-Johnson syndrome. In Stevens-Johnson syndrome, mucosal involvement of the lip (93%), conjunctiva (82%), oral cavity (79%), and nose (36%) were most common. Antecedent medication use was identified in 59% of erythema multiforme patients and 68% of Stevens-Johnson syndrome patients. We note a striking increase in the number of cases in our series caused by cephalosporins. Fifty percent of Stevens-Johnson syndrome patients required supplemental hydration or alimentation because of the severity of the oral cavity involvement. The head and neck mucosal manifestations largely respond to local care, and the routine use of prophylactic antibiotics or systemic steroids is not recommended.

Immunohistochemical Expression of Apoptotic Markers in Drug-Induced Erythema Multiforme, Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

2007 ◽  
Vol 20 (3) ◽  
pp. 557-566 ◽  
Author(s):  
A.V. Marzano ◽  
A. Frezzolini ◽  
M. Caproni ◽  
A. Parodi ◽  
D. Fanoni ◽  
...  
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
Erythema Multiforme ◽  
Fas Ligand ◽  
Normal Skin ◽  
Basal Layer ◽  
Immunohistochemical Analysis ◽  
Stevens Johnson Syndrome ◽  
Drug Induced ◽  
Johnson Syndrome ◽  
Dermal Infiltrate

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered to be severity variants of the same disease, which is almost always associated with drug intake. In contrast, erythema multiforme (EM) is a disorder regarded as only rarely caused by drugs. Keratinocyte apoptosis has been shown to play an important part in the pathogenesis of SJS and TEN, whilst its role in EM remains controversial. To determine the expression of apoptosis-associated molecules Fas, Fas ligand (FasL), Bcl-2 and Bax in the above disorders, an immunohistochemical analysis was performed. We studied both lesional skin from thirty patients having drug-induced EM and 5 cases classified within the SJS/TEN spectrum and normal skin samples. We found a keratinocyte overexpression of Fas antigen, an important molecule mediating apoptosis, not only in SJS and TEN but also in EM. Another noteworthy finding was the strong expression of Bcl-2, a protein known as blocking apoptosis, along the basal layer and in the dermal infiltrate both in SJS/TEN and in EM. Taken together, these findings suggest that Fas-dependent keratinocyte apoptosis may play a part in the pathogenesis of both SJS/TEN and EM. Fas-mediated cell death may be partially suppressed by the Bcl-2 protein.

scholarly journals A clinical study of Stevens-Johnson syndrome and toxic epidermal necrolysis in a tertiary centre, South India

2017 ◽  
Vol 3 (1) ◽  
pp. 134
Author(s):  
Rahima S. ◽  
Abdul Latheef E. N. ◽  
Pavithran K. ◽  
Saleem P. M.
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
South India ◽  
Detailed Examination ◽  
Stevens Johnson Syndrome ◽  
Hiv Positive ◽  
Tertiary Centre ◽  
Johnson Syndrome ◽  
Mucosal Involvement ◽  
Common Causes ◽  
Eye Involvement

<p class="abstract"><strong>Background:</strong> Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are considered as the severest end of spectrum of erythema multiforme. Various etiologies like infections, drugs and malignancies have been proposed. The aim of the present study was to know the incidence, common causes, clinical course of SJS and TEN and to estimate the morbidity and mortality<span lang="EN-IN">.</span></p><p class="abstract"><strong>Methods:</strong> A 2 year study of patients presenting with SJS and TEN was carried out. A detailed examination to know the cutaneous and mucosal involvement was done. Biopsy was done in 3 patients.<strong></strong></p><p class="abstract"><strong>Results:</strong> There were fifty patients of SJS-TEN spectrum. Of which 31 were SJS, 3 had SJS-TEN overlap and 16 had TEN.  Anticonvulsants were implicated in causing these reactions in 24 patients (48%) with carbamazepine being the most common i.e. in 16 patients (32%). Sparing of pressure areas like the strap area of brassier and waist was noticed in two patients (4%). The most common complication was due to eye involvement seen in 20 patients (40%). 46 patients were treated with steroids and of the remaining, 3 were children and one was HIV positive. Only three patients with TEN (6%) died<span lang="EN-IN">. </span></p><p class="abstract"><strong>Conclusions:</strong> To conclude, TEN was less common than SJS, had more sequelae and more mortality compared to SJS<span lang="EN-IN">.</span></p><p class="abstract"> </p>

scholarly journals Toxic Epidermal Necrolysis and Stevens-Johnson Syndrome Are Induced by Soluble Fas Ligand

2003 ◽  
Vol 162 (5) ◽  
pp. 1515-1520 ◽  
Author(s):  
Riichiro Abe ◽  
Tadamichi Shimizu ◽  
Akihiko Shibaki ◽  
Hideki Nakamura ◽  
Hirokazu Watanabe ◽  
...  
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
Fas Ligand ◽  
Stevens Johnson Syndrome ◽  
Soluble Fas ◽  
Johnson Syndrome ◽  
Soluble Fas Ligand

scholarly journals Patients with Stevens - Johnson syndrome and Toxic Epidermal Necrolysis Develop Coagulopathies Similar to Those Seen in Burn Patients: A Pilot Study

2018 ◽  
Vol 3 (1) ◽  
Keyword(s):  
Toxic Epidermal Necrolysis ◽  
Plasminogen Activator ◽  
Interleukin 6 ◽  
Plasma Levels ◽  
Total Body Surface Area ◽  
Disease Process ◽  
Stevens Johnson Syndrome ◽  
Burn Patients ◽  
Johnson Syndrome ◽  
D Dimer

Background: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are a continuum of a life-threatening skin loss condition due to an immune or hypersensitivity reaction. Patients are frequently treated in burn centers. Objective: This study was undertaken to determine if patients with SJS and TEN have a coagulopathy with comparable hemostatic perturbations to those seen in patients with burn injury. Materials & Methods: Blood plasma parameters studied were factors of coagulation, fibrinolysis, Interleukin-6 (IL-6) and Endothelin-1. Results were compared to historical hemostatic and cytokine data from burn patients treated at the same center. Results: Sixteen patients with SJS-TEN (6 males/10 females) with ≥20 % total body surface area (TBSA) sloughed skin were studied. The majority had received phenytoin or an antibiotic as the precipitating medication for the SJS-TEN. There was a significant increase in Thrombin-Antithrombin Complex (TAT) p<0.0004, tissue Plasminogen Activator (tPA), p<0.02, Plasminogen Activator Inhibitor-1 (PAI-1), p<0.02, and D-dimer p<0.007 plasma levels on admission. Antithrombin (AT), p<0.04 and plasminogen (PLG) p<0.02 plasma levels were significantly decreased. Conventional global coagulation tests (prothrombin and partial thromboplastin times) were not abnormal in patients with ≤7 days duration of the rash on admission. Patients with delayed admission at >7 days after the start of the rash had a significantly increased chance of demise, p<0.01. These patients also had a significantly decreased AT levels (p<0.01) compared to normal controls and to patients admitted at ≤7 days of the disease process, (p<0.01). The pattern of hemostatic aberrations of TAT, tPA, PAI1, D-dimer, Interleukin -6, AT, and PLG was similar to that seen in burn patients during the acute phase of injury and resuscitation. The mortality rate was 37.5 %. Conclusions: Patients with ≥20% TBSA SJS-TEN had hemostatic perturbations consistent with those observed in ≥20% TBSA burn injuries coagulopathies.

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