Stevens-Johnson Syndrome Medical Literature

 

Risks of Stevens Johnson Syndrome (SJS / TEN)

Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) may be fatal

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).

 

Received 13 July 2009; accepted 2 October 2009.

DIGITAL OBJECT IDENTIFIER (DOI)

10.1111/j.1346-8138.2009.00746.x About DOI

 

Dermatology Online Journal

"Severe skin adverse drug reactions can result in death. Toxic epidermal necrolysis (TEN) has the highest mortality (30-35%); Stevens-Johnson syndrome and transitional forms correspond to the same syndrome, but with less extensive skin detachment and a lower mortality (5-15%)..." etc.

http://dermatology.cdlib.org/DOJvol8num1/reviews/drugrxn/ghislain.html

Wilmer Eye Institute at Johns Hopkins  Conditions

"Stevens-Johnson Syndrome (SJS) is a disorder that causes painful blisters and lesions on the skin and mucous membranes and can cause severe eye problems. The most common cause of SJS is an adverse allergic drug reaction......"

http://www.hopkinsmedicine.org/wilmer/Conditions/stevens-johnson.html

Stevens-Johnson syndrome and toxic epidermal necrolysis are often drug induced

"Toxic epidermal necrolysis (TEN) is an acute dermatologic disease, the presentation of which may constitute a true emergency. The disorder is characterized by widespread erythematous macules and targetoid lesions; full-thickness epidermal necrosis, at least focally; and involvement of more than 30% of the cutaneous surface. Commonly, the mucous membranes are also involved. Nearly all cases of toxic epidermal necrolysis are induced by medications, and the mortality rate can approach 40%"......

http://emedicine.medscape.com/article/1124127-overview

Skin Diseases Fritsch P; European Dermatology Forum: skin diseases in Europe. Skin diseases with a high public health impact: toxic epidermal necrolysis and Stevens-Johnson syndrome. Eur J Dermatol. 2008 Mar-Apr;18(2):216-7.
The risk of Stevens-Johnson syndrome and toxic epidermal necrolysis associated with nonsteroidal antiinflammatory drugs: a multinational perspective

"The risk of the severe cutaneous adverse reactions Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) associated with use of nonsteroidal antiinflammatory drugs (NSAID). ".....

http://www.jrheum.org/content/30/10/2234.abstract

Stevens Johnson Syndrome: A Case Report

"Stevens-Johnsonsyndrome(SJS)isalife-threateningvesicu-
lobullous diseasecharacterizedbyanacuteeruptionthat
involves theskinandmucousmembranes.Variousetiologic
factorshavebeenimplicatedasacauseofSJS,including
infection,vaccination,drugs,systemicdiseases,physical
agents, andfood.Drugsarethemostcommonlyblamed.".......

 

An update on Stevens-Johnson syndrome and toxic epidermal necrolysis in children

"Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) and provides an update on the treatment of these conditions in children.".......

Newer agents such as cyclosporin, infliximab and plasmapheresis have shown promise in the treatment of SJS and TEN.

Drugs associated with Stevens-Johnson Syndrome in Children

http://www.nelm.nhs.uk/en/NeLM-Area/News/2009---February/09/Drugs-associated-with-Stevens-Johnson-syndrome-in-children/

 

Lars E French, 2006 Allergology International

 

 

"Toxic Epidermal Necrolysis and
Stevens Johnson Syndrome: Our
Current Understanding"; Allergology International Vol 55, No1, 2006 http: www.jsaweb.jp

 

Mockenhaupt M., et al., Jour of Rheumatology, 2003 "The Risk of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Associated with Nonsteroidal Antiinflammatory Drugs: A Multinational Perspective," Vol. 30, No. 10, pp. 2234-40
Roujeau J.C., et al., New England Jour of Med, 1995

"Medication Use and the Risk of Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis," Vol. 333, No. 24, pp. 1600-7

 

Tadamasa Yoshitake, 2007

Erythema multiforme and Stevens-Johnson syndrome following radiotherapy" Radiat Med (2007) 25:27�30 DOI 10.1007/s11604-006-0093-3

Fever and rash in a child: when to worry? The differential diagnosis of a fever and rash presenting in a pediatric patient is quite extensive. This article is not all-inclusive but is meant to aid in the diagnosis to differentiate serious, life-threatening eruptions from more benign common rashes.

Causes of Stevens Johnson Syndrome (SJS / TEN)

Mockenhaupt M., et al., Jour of Rheumatology, 2003

"The Risk of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Associated with Nonsteroidal Antiinflammatory Drugs: A Multinational Perspective," Vol. 30, No. 10, pp. 2234-40
Ariane Auquier-Dunant, ArchDermatol. 2002;13;1019-1024 "Correlations Between Clinical Patterns and Causes of Erythema Multiforme Majus, Stevens-Johnson Syndrome, and Toxic Epidrmal Necrolysis", style="text-decoration: none">Arch Dermatol Vol 138, Aug 2002
CRAIG K. SVENSSON, 2000 Pharmacol Rev 53:357�379

Cutaneous Drug Reactions  

PHARMACOLOGICAL REVIEWS Vol. 53, No. 3

Copyright � 2000 by The American Society for Pharmacology and Experimental Therapeutics 131/927786

Roujeau J.C., et al., New England Jour of Med, 1995 "Medication Use and the Risk of Stevens-Johnson Syndrome or Toxic Epidermal Necrolysis," Vol. 333, No. 24, pp. 1600-7

Brkljaci 2006

Brkljaci N, Gracin S, Prkacin I, Sabljar-Matovinovi? M, Mrzljak A, Nemet Z. Stevens-Johnson syndrome as an unusual adverse effect of azithromycin. Acta Dermatovenerol Croat. 2006;14(1):40-5. [view PubMed record (16603101)]
 


Stevens Johnson Syndrome (SJS / TEN) Treatment and Outcomes

American College of Emergency Physicians, 2006 IVIG Benefits Some Children With Stevens-Johnson Syndrome
 
Stevens Johnson Syndrome Foundation Treatments from Stevens Johnson Syndrome Foundation about eyes / eye sight / lens
Haber, 2004 Late Outcomes in Adult Survivors of Toxic Epidermal Necrolysis After Treatment in a Burn Center
Ducic, 2002

Plastic and Reconstructive Surgery 
"Outcome of Patients with Toxic Epidermal Necrolysis Syndrome Revisited", Vol. 110, No. 3, pp. 768 - 73
Pierre-Dominique Ghislain M.D., 2002 Dermatol Online J 8(1), 2002

"Treatment of Severe Drug Reactions" -- Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis;  Department of Dermatology, Hospital Henri Mondor, University Paris XII, Creteil, France

"Improving the Outcome of Patients
With Toxic Epidermal Necrolysis
and Stevens-Johnson Syndrome"
Garcia-Doval, I, et. al., Arch Dermatol, 2000 "Toxic Epidermal Necrolysis and Stevens Johnson Syndrome - Does early withdrawal of Causative Drugs Decrease the Risk of Death"; Arch Dermatol 2000, 136:323-327
Facts on Prevention, Detection & Treatment from John Hopkins Medicine Cutaneous eruptions are the most frequently reported adverse reactions to drugs, and most medications have a 1% to 3% risk of producing immunologic manifestations. Drug-induced allergic reactions occur in approximately 5% of the population, and these reactions are responsible for approximately 6% to 10% of all adverse drug reactions. Of all medication classes, b-lactam antibiotics, sulfonamides, and nonsteroidal anti-inflammatory drugs (NSAIDs) are responsible for 80% of all reports of allergic and pseudoallergic reactions.

The Spectrum of Drug-Induced Cutaneous Adverse Reactions


Early Recognition & Warning Signs of Severe Skin Reactions


Discussion of Cross-Reactivity & Drug Induced Cutaneous Reactions Among Highly Prescribed Drug Classes

Appropriate Treatment of Severe Skin Reactions: SJS/TEN
 

Searching for Answers about Allergic
Reactions and Antiseizure Medications

However, carbamazepine, lamotrigine and phenytoin were associated with
higher rates of rash. Oxcarbazepine and phenobarbital had similar rates, but the
number of patients receiving these agents was less, slightly eroding the confidence in these numbers.

http://www.columbiaepilepsy.org/docs/patients/PN0905EpilepsyEssentials.pdf

Chang YS, 2007 Chang YS, Huang FC, Tseng SH, Hsu CK, Ho CL, Sheu HM. Erythema multiforme, Stevens-Johnson syndrome, and toxic epidermal necrolysis: acute ocular manifestations, causes, and management. Cornea. 2007 Feb;26(2):123-9. [view PubMed record (17251797)]
Ang LP 2007 Ang LP, Sotozono C, Koizumi N, Suzuki T, Inatomi T, Kinoshita S. A comparison between cultivated and conventional limbal stem cell transplantation for Stevens-Johnson syndrome. Am J Ophthalmol. 2007 Jan;143(1):178-80. [view PubMed record (17188066)
Clayton NA 2007 "Management of Dysphagia in Toxic Epidermal Necrolysis (TEN) and Stevens-Johnson Syndrome (SJS)."
Burns. 2007 Apr 30; [Epub ahead of print] Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS): Experience with high-dose intravenous immunoglobulins and topical conservative approach A retrospective analysis.
Dermatol Clin. 2007 Apr;25(2):245-53. Recognition and management of severe cutaneous drug reactions.

What is Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis?

Laguna C 2006 Laguna C, Martn B, Torrijos A, Garc�a-Melgares ML, Febrer I. [Stevens-Johnson syndrome and toxic epidermal necrolysis] Actas Dermosifiliogr. 2006 Apr;97(3):177-85. [view PubMed record (16796964)]