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Johnson Syndrome (johnson + syndrome)

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Medical Sciences	100%

Selected Abstracts

Treatment of erythema multiforme, Stevens,Johnson Syndrome, and toxic epidermal necrolysis

DERMATOLOGIC THERAPY, Issue 4 2002
Klemens Rappersberger
The "erythema multiforme disease spectrum" comprises four distinct, severe, clinical subvariants: (1) bullous erythema multiforme (bullous-EM), (2) Stevens,Johnson syndrome (SJS), (3) SJS,toxic epidermal necrolysis (TEN)-overlap syndrome, and (4) TEN. These diseases are closely related to severe mucocutaneous intolerance reactions that are mostly elicited by drugs/drug metabolites and associated with a high mortality rate. Old age and area of detached skin negatively influence the course of disease, and early withdrawal of causative drugs with short half-life is a positive prognostic factor. Therapeutic management represents a multidisciplinary challenge for colleagues from various specialities including specialized nurses and usually can be performed at a dermatologic ward unless technical equipment of an intensive care unit is needed. Topical therapy with biologic and (semi-)synthetic dressings is aimed at early re-epithelialization and the prevention of scarring, synechia formation, and infection. Systemic treatment includes antibiotics, fluid and electrolyte replacement, protein preparations and blood products, etc. Various anti-inflammatory and immunosuppressive treatment regimens with corticosteroids, cyclosporine A, cyclophosphamide, plasmapheresis have been considered to halt ongoing immunologic pathomechanisms, and some of these have shown significant efficacy. However, because we lack formal clinical trials, none of these regimens can be definitively proposed as a therapy of choice in any of the severe clinical variants of the EM spectrum. [source]

Stevens,Johnson Syndrome: A Diagnostic Challenge in the Absence of Skin Lesions

PEDIATRIC DERMATOLOGY, Issue 1 2003
Inge Vanfleteren M.D.
Stevens,Johnson syndrome in children is most frequently caused by a Mycoplasma pneumoniae infection. The full clinical picture of Stevens,Johnson syndrome can be present before seroconversion of Mycoplasma antibodies is observed. One should keep in mind that one negative titer of Mycoplasma antibodies does not rule out M. pneumoniae infection. [source]

Utilization of hospital and outpatient care for adverse cutaneous reactions to medications

PHARMACOEPIDEMIOLOGY AND DRUG SAFETY, Issue 10 2005
Robert S. Stern MD
Abstract Purpose To quantify hospitalizations, visits to office based physicians, hospital clinics and emergency departments with primary diagnoses of skin conditions that are often due to drug reaction. Methods I analyzed data from the National Hospital Discharge Summary (1997,2001), National Ambulatory Care Survey (1995,2000) and National Hospital Ambulatory Care Survey (1995,2000) to determine the number of hospitalizations and visits with primary diagnoses of skin conditions that are often attributed to drugs. Using statistical methods for surveys, I determined the demographic characteristics of patients with these diagnoses and compared them with patients seeking care for other reasons. Results In the United States, there are about 5000 hospitalizations each year with a primary diagnosis of erythema multiform, Stevens,Johnson Syndrome or Toxic Epidermal Necrolysis, of which 35% are specifically ascribed to drugs. Annually, there are more than 100,000 outpatient visits for these diagnoses and about two million visits for immediate hypersensitivity reactions that may be due to drugs. Outpatient visits for drug eruptions and drug allergies that include a skin component exceed 500,000 annually. Conclusions Skin conditions often attributed to drugs are frequent reasons for hospitalization and physician visits. Optimal care of the individual patients with these conditions requires careful attention to drugs as a possible cause. Copyright © 2005 John Wiley & Sons, Ltd. [source]

Nevirapine-Induced Stevens Johnson,Syndrome and Fulminant Hepatic Failure Requiring Liver Transplantation

AMERICAN JOURNAL OF TRANSPLANTATION, Issue 7 2010
J. Jao
We describe a case of nevirapine-induced Stevens,Johnson Syndrome (SJS) and fulminant hepatic failure (FHF) requiring liver transplantation. Five weeks prior to admission, a 57-year-old female with HIV infection had been switched to a nevirapine-based regimen of highly active antiretroviral therapy (HAART) with a CD4 cell count of 695/mm3. Examination of the explanted native liver at initial transplantation revealed massive hepatic necrosis consistent with drug-induced liver injury. Primary graft nonfunction complicated the early postoperative course and liver retransplantation was required. On follow-up 2 years later, she remains in good health with an undetectable viral load on an efavirenz-based regimen of HAART. To our knowledge, this is the first report of successful liver transplantation following SJS and FHF. [source]

The Boston keratoprosthesis in autoimmune disease

ACTA OPHTHALMOLOGICA, Issue 2009
J CHODOSH
Purpose Patients with corneal blindness due to mucous membrane pemphigoid and Stevens Johnson syndrome who undergo corneal transplantation carry a poor prognosis for visual recovery. The Boston keratoprosthesis has been demonstrated to provide excellent retention rates and postoperative visual acuity in patients with corneal graft failure, however, poor visual outcomes still occur in patients with underlying autoimmune disease. Methods We reviewed the current literature to determine the results of keratoprosthesis in patients with blinding autoimmune diseases. Results Much of the published literature on keratoprosthesis fails to clearly differentiate outcomes on the basis of the underlying disorder. Based on available evidence, inflammation, retinal detachment, and glaucoma appear to be the most significant complications after keratoprosthesis in autoimmune patients, and a diagnosis of mucous membrane pemphigoid or Stevens Johnson Syndrome appears to be associated with a significantly higher complication rate than other preoperative conditions. Conclusion Patients with autoimmune diseases carry the worst prognosis for success with keratoprosthesis. Improvement in clinical outcomes might be achieved with changes in keratoprosthesis design and material, perioperative therapy, and/or surgical technique. Possible approaches to complications after Boston keratoprosthesis in patients with underlying autoimmune diseases will be discussed. [source]

Tetrazepam drug sensitivity , usefulness of the patch test

CONTACT DERMATITIS, Issue 3 2002
C. Pirker
The muscle relaxant tetrazepam may cause severe cutaneous adverse effects. We report 4 cases of varying intensity: Stevens,Johnson syndrome, erythema,multiforme-like exanthema, maculopapular and maculo-urticarial exanthema. Patch testing with tetrazepam (10% in petrolatum) was strongly positive in the 2 patients with severe skin eruptions and weakly positive in the other 2. Oral rechallenge with tetrazepam was positive in 3 patients (1 not done). Diazepam, with a similar chemical structure to tetrazepam, was negative on patch testing and on oral challenge testing in 2 patients. Although the optimal patch test concentration of tetrazepam has still to be determined, it is a useful diagnostic tool to confirm sensitization, particularly in patients with severe bullous eruptions. [source]

Treatment of erythema multiforme, Stevens,Johnson Syndrome, and toxic epidermal necrolysis

Genetic background of Japanese patients with adult-onset storage diseases in the liver

HEPATOLOGY RESEARCH, Issue 10 2007
Hisao Hayashi
In contrast to primary lysosomal diseases in young subjects, adult-onset liver storage disorders may be explained by non-lysosomal genetic defects. The aim of the present review is to summarize the genetic backgrounds of Japanese patients with hemochromatosis of unknown etiology, Wilson disease of primary copper toxicosis, and the black liver of Dubin,Johnson syndrome. Three patients with middle-age onset hemochromatosis were homozygous for mutations of HJV and two patients were homozygous for mutations of TFR2. Minor genes other than HJV and TFR2 might be involved in Japanese patients. Five of the six patients with Wilson disease were compound heterozygous, while the remaining patient was heterozygous for the mutation in ATP7B responsible for copper toxicosis. Involvement of MURR1 was not proved in the heterozygote of ATP7B. Because of ferroxidase deficiency,most patients had secondary lysosomes shared by cuprothioneins and iron complex. Six patients with Dubin,Johnson syndrome were homozygous or compound heterozygous for mutant MRP2. Despite complex metabolic disorders, the syndrome had a single genetic background. Thus, most patients with adult-onset lysosomal proliferation in the liver had genetic defects in non-lysosomal organelles, named the secondary lysosomal diseases. The proliferating lysosomes in these conditions seemed to be heterogeneous in their matrices. [source]

Severe cutaneous reactions caused by barbiturates in seven Iranian children

INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 11 2009
Setareh Mamishi MD
Background, The severe adverse cutaneous reactions of erythema multiforme (EM), Stevens,Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare mucocutaneous diseases associated with significant morbidity and mortality. The most common cause is antiepileptic drugs, particularly carbamazepine and lamotrigine, as well as the barbiturates group (phenobarbital and phenytoin). In this article, we present seven children with severe adverse cutaneous reactions caused by barbiturates. Case Reports, The age of the affected children was between 2 and 11 years and they all had a history of taking barbiturates. Their symptoms started 1,3 weeks after the initiation of barbiturates, including a prodrome characterized by 2,3 days of malaise, fever, cough and anorexia, after which the skin and mucosal lesions appeared and worsened. The skin lesions varied from rash to large bullae, plus different forms of mucous membrane involvement. The offending drugs (barbiturates) were stopped immediately and care was largely supportive. Conclusion, As a result of the morbidity and/or mortality associated with EM, SJS and TEN, physicians should keep in mind their differential diagnosis when cutaneous reactions are observed in patients undergoing barbiturate therapy. Furthermore, although TEN and SJS are life-threatening diseases, early detection and appropriate care can lead to a decrease in the incidence of death. The strategies described here seem to be successful and safe because, despite the serious conditions, our patients responded well. All survived. [source]

Stevens,Johnson syndrome after amifostine during radiotherapy

INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 4 2004
Leonardo Astudillo MD
No abstract is available for this article. [source]

Reintroducing antituberculosis therapy after Stevens,Johnson syndrome in human immunodeficiency virus-infected patients with tuberculosis: role of desensitization

INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 7 2001
Mahendra M. Kura MD
First page of article [source]

Stevens,Johnson syndrome due to mirtazapine , first case

ALLERGY, Issue 10 2009
A. Belkahia
No abstract is available for this article. [source]

Salivary flow and its relationship to oral signs and symptoms in patients with dry eyes

ORAL DISEASES, Issue 2 2004
M Koseki
Objectives:, The aim of this study was to investigate oral symptoms and clinical parameters in dry eye patients. Subjective reports of the sensation of a dry mouth, salivary flow rates, and clinical parameters of oral disease related to three different types of dry eye patients were examined. Subjects and methods:, There were 224 individuals, including dry eye patients and control subjects. The dry eye patients were classified into three types: patients with Sjögren's syndrome (SS-DE), patients without SS-DE (non-SS-DE), and patients with Stevens,Johnson syndrome (SJS-DE). Salivary flow rates were measured using two kinds of sialometry. Subjective and objective oral symptoms and signs were also examined. Results and conclusion:, Over half of the dry eye patients complained of a dry mouth. The flow rates of their stimulated whole saliva and parotid saliva were significantly lower than those of the control groups (P < 0.05, P < 0.01). The sensation of a dry mouth and changes in oral soft tissues, dental caries, and oral Candida frequently occurred in dry eye patients. [source]

Paraneoplastic Pemphigus with Bronchiolitis Obliterans in a Child

PEDIATRIC DERMATOLOGY, Issue 3 2003
M.D., Winnie A. Mar
Most cases have been reported in adults and the number of childhood cases in the current literature is limited. We describe a young patient with PNP who was initially misdiagnosed as having recurrent Stevens,Johnson syndrome. This patient had an underlying inflammatory myofibroblastic tumor and subsequently developed fatal progressive bronchiolitis obliterans. [source]

Stevens,Johnson Syndrome: A Diagnostic Challenge in the Absence of Skin Lesions

Erythema multiforme, Stevens,Johnson syndrome and toxic epidermal necrolysis: Frozen-section diagnosis

THE JOURNAL OF DERMATOLOGY, Issue 5 2010
Hiroomi HOSAKA
Abstract Stevens,Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) may be fatal. Although classified by body surface area skin detachment, initial stages of both may present with erythema multiforme (EM)-like lesions. To diagnose and predict disease activity adequately as early as possible for patients revealing EM-like lesions, we performed frozen-section diagnosis. Thirty-five patients clinically diagnosed as EM, SJS or TEN were biopsied to diagnose and predict disease progression within the initial-visit day. Half of a histological section taken from a lesion was snap-frozen and immediately cryostat-sectioned, acetone-fixed and stained with hematoxylin,eosin. Specimens were examined with light microscopy for presence of epidermal necrosis. A section from unaffected sites was also examined for 11 patients. Specimens were examined with light microscopy for presence of graft-versus-host reaction (GVHR)-like findings: apoptotic keratinocytes and satellite cell necrosis. Epidermal necrosis was seen in nine patients. Initial diagnosis of the nine was one of overlap SJS-TEN, four of SJS and four of EM, and final diagnosis of those was one of TEN, one of overlap SJS,TEN, four of SJS and three of EM. Dissociation between initial and final diagnosis was seen in three cases. GVHR-like findings in the epidermis were observed in two patients finally diagnosed as overlap SJS,TEN and TEN. Frozen sections are useful not only to make a diagnosis of erythema multiforme but to assess a potential to exhibit more aggressive clinical behaviors (SJS or TEN). [source]

Open trial of ciclosporin treatment for Stevens,Johnson syndrome and toxic epidermal necrolysis

BRITISH JOURNAL OF DERMATOLOGY, Issue 4 2010
L. Valeyrie-Allanore
Summary Background, Stevens,Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute mucocutaneous reactions associated with poor prognosis. The treatment is mainly symptomatic, based on supportive care. Until now, several curative treatments have been proposed without evidence of effectiveness. Objectives, To evaluate the effect of ciclosporin on SJS and TEN after a short series had suggested a benefit. Methods, We conducted an open, phase II trial to determine the safety and possible benefit of ciclosporin. Among the 45 consecutive patients admitted for SJS/TEN from March 2005 to September 2007, 29 fulfilled inclusion criteria. Ciclosporin was administered orally (3 mg kg,1 daily for 10 days) and tapered over a month. Clinical and biological evaluations were performed sequentially. Predicted death rate was estimated with a validated prognostic score (SCORTEN). Results, Twenty-nine patients were included at a mean ± SD of 2·8 ± 1·8 days after onset. The final diagnosis was SJS (n = 10), SJS/TEN overlap (n = 12) and TEN (n = 7). One month of treatment was completed in 26. Ciclosporin was stopped after more than 10 days in three cases for side-effects including posterior leucoencephalopathy (n = 1), neutropenia (n = 1) and nosocomial pneumopathy (n = 1). Ciclosporin dosage was tapered earlier than scheduled in two cases for alteration in renal function. The prognostic score predicted 2·75 deaths; none occurred (P = 0·1). Mean epidermal detachment remained stable in 18 of 29 cases (62%). The mean ± SD hospital stay was 16·2 ± 9·1 days. Conclusions, Both the death rate and the progression of detachment seemed lower than expected, suggesting a possible usefulness of ciclosporin in SJS and TEN that needs to be confirmed. [source]

Two cases of Stevens,Johnson syndrome: toxic epidermal necrolysis possibly induced by amifostine during radiotherapy

BRITISH JOURNAL OF DERMATOLOGY, Issue 5 2000
. Lale Atahan
No abstract is available for this article. [source]

The Boston keratoprosthesis in autoimmune disease

How do we get started with offering MOOKP clinical service?

ACTA OPHTHALMOLOGICA, Issue 2009
M FUKUDA
Modified osteo-odonto keratoprosthesis (MOOKP) is complicated two step surgery. Firstly, we must understand why it is effective for visual recovery of end-stage ocular surface diseases like Stevens- Johnson syndrome. MOOKP have a lot of advantages compared to other K-pros, for example the using auto tissue of canine tooth root and buccal mucous membrane, the tight adhesions between optical cylinder and canine tooth root, the adhesion between MOOKP lamina and sclera or cornea, the strong ocular surface by auto buccal mucous membrane, no inflammation on the back of optical cylinder and so on. However, the precise surgical techniques and proper instructions are necessary to succeed the very first case in newly set surgical center. In Japan, we successfully set up the MOOKP center and did perform 4 cases of MOOKP since 2003. We share our experience about it and point out our modification adjustable for Japanese patients. [source]