Epilepsy Foundation

I have a new auto-immune disease called Stevens-Johnson Syndrome.  No one has heard of it when it is mentioned.  It is deadly with no treatment.  MIne is a reation to antibiotics or possibly other meds.  I have had 3 reactions (1st 2 undiagnosed) all 3 happened after 3 diff. antibiotics. Symptoms: mouth full of ulcers, 4 ulcers on foot and legs, other body ulcers, very painful, starts with rapid breathing and break out all over trunk area, closing of throat, chills, fever, many more, my eyes effected too.  It is treated like you are a burn patient.  Some of the photos are grusome, however mine is mild and I pray it stays that way.  I am still not healed and it has been since Jan 18.  I am questioning how will this effect my E and my meds.  I have been to the ER twice, and several different types of doctors.  My neuro doesn't want to change any of my meds because my seizures are under control however he is very concerned.  Any info will be helpful.  God bless you all.  Melissa

physicians seen SJS: neurologist, rheumatologist, internist, dermotologist, allergist, opthamologist.  This is such a complicated disorder took so many docs and I feel like a science experimental case when I go in.  They have never seen an actual person with this. 


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Hi Melissa,
Sorry you are dealing with SJS, I hope you get better soon.

SJS is a well known dangerous side effect of many drugs and infections and for a bunch of DR's to not know about SJS is unacceptable.

Hi Melissa,

my name is Gigi and I am an Information Specialist with the Epilepsy Foundation's Epilepsy Resource Center.  I thought that maybe this link will answer some of your questions:

http://www.epilepsyfoundation.org/about/treatment/medications/medso...

There are also some very interesting articles available on the EF website that may be of interest to you. Go to www.epilepsyfoundation.org and in the search box type Stevens Johnson Syndrome, click on the headings (1) Curing Epilespy and (2) Current approaches........

 

We look forward to hearing from you again in the near future.

 

Cordially,

Gigi Jones

Information Specialist

In response to your inquiry, Stevens- Johnson syndrome (SJS) occurs in 1:5000-10 000 patients exposed to carbamazepine, phenytoin, phenobarbitone or lamotrigine. (Atlas of epilepsy, Appleton RE (ed) p 118). From the abstracts below, it appears that patients who are at a higher risk for SJS are those with another AED rash, as well as new users of drugs, and there may be genetic causes as well.
For more information about the risks, causes and treatment of SJS, please contact Cecille Jach, Administrator, National Epilepsy Library®, at 1-800-332-4050ext 3737, or cjach@efa.org.

(31455) 2007 - Comparison and predictors of rash associated with 15 antiepileptic drugs.
Neurology. 2007;68(20):1701-9.
Arif H, Buchsbaum R, Weintraub D, et al
OBJECTIVE: To determine predictors and relative incidence of antiepileptic drug (AED)-related rash in patients taking all common AEDs. METHODS: We reviewed 1,890 outpatients. Eighty-one variables were tested as potential predictors of rash. We compared the rate of rash attributed to each AED (AED rash) with the average rate of rash attributed to the other AEDs in all adults (aged > or =16 years; n = 1,649) when taking carbamazepine (CBZ), clobazam (CLB), felbamate (FBM), gabapentin (GBP), lamotrigine (LTG), levetiracetam (LEV), oxcarbazepine (OXC), phenobarbital (PB), phenytoin (PHT), primidone (PRM), tiagabine (TGB), topiramate (TPM), vigabatrin (VGB), valproate (VPA), or zonisamide (ZNS). We repeated this analysis for patients with and without the identified nondrug predictors of AED rash. RESULTS: The average rate of AED rash was 2.8%. The only nondrug predictor significant in multivariate analysis was occurrence of another AED rash (odds ratio 3.1, 95% CI 1.8 to 5.1; p < 0.0001); the rate of rash in this subgroup was 8.8%, vs 1.7% in those without another AED rash. Higher AED rash rates were seen with PHT (5.9% overall, p = 0.0008; 25.0% in those with another AED rash, p = 0.001), LTG (4.8%, p = 0.00095; 14.4%, p = 0.025), and CBZ (3.7%, not significant; 16.5%, p = 0.01). Lower rates were seen with LEV (0.6% overall; p = 0.00042), GBP (0.3%, p = 0.00035), and VPA (0.7%, p = 0.01). Rash rates were also low (<1% overall) with FBM, PRM, TPM, and VGB (not significant). These AED differences remained similar in patients with and without other AED rashes. There were four cases of Stevens-Johnson syndrome involving four AEDs. CONCLUSIONS: The rate of an antiepileptic drug (AED) rash is approximately five times greater in patients with another AED rash (8.8%) vs those without (1.7%). Rash rates were highest with phenytoin, lamotrigine, and carbamazepine and low (<1%) with several AEDs
(31471) 2006 - Treatment of anticonvulsant hypersensitivity syndrome with intravenous immunoglobulins and corticosteroids.
: J Child Neurol. 2006;21(5):380-4.
Prais D, Straussberg R, Amir J, et al
Anticonvulsant hypersensitivity syndrome is a specific severe idiosyncratic reaction to the aromatic antiepileptic drugs. The most frequent presenting symptoms are fever and rash, lymphadenopathy owing to lymphoid hyperplasia, hepatitis, and interstitial nephritis. Severe skin reactions (Stevens-Johnson syndrome or toxic epidermal necrolysis) have also been reported. Early detection is crucial owing to the high mortality rate. Although withdrawal of the offending drug is critical, the optimal treatment approach remains controversial. Previous studies report a severe course and prolonged hospital stay for cutaneous drug-related reactions, including referral to a burn center, skin débridement, and allograft skin coverage. The aim of the present report is to describe four adolescents with antiepileptic drug hypersensitivity syndrome who were treated with intravenous immunoglobulin and systemic corticosteroids. All recovered completely following an uncomplicated and relatively short course and hospitalization. Findings indicate that this regimen might be a promising treatment option in this patient population. Larger, controlled trials are needed to reach a definitive conclusion.
(32604) 2005 - Risk of Stevens-Johnson syndrome and toxic epidermal necrolysis in new users of antiepileptics.
Neurology. 2005;64(7):1134-8.
Mockenhaupt M, Messenheimer J, Tennis P, et al
BACKGROUND: Estimates of risk of Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) associated with some antiepileptic drugs (AEDs) have used denominators based on the number of prescriptions or daily doses. Because the risk of SJS is highest in new users of drugs, the use of denominators reflective of all users can lead to low estimates of risk associated with drugs. In this study, risk in new users is assessed. METHODS: Data on all hospitalized patients with SJS and TEN with use of carbamazepine (CBZ), lamotrigine (LTG), phenobarbital (PHB), phenytoin (PHT), or valproic acid (VPA) were obtained from the German Registry for Serious Cutaneous Reactions. For 1998-2001, the numbers of new users were estimated from number of dispensed prescriptions in Germany, the average prescribed doses, and the duration of use in the Mediplus database (IMS Health) Germany, and assumptions that relate new use to growth in national dispensings. To minimize the probability of underestimating risk in new users, conservative estimates of new use that were somewhat lower than predicted from national prescription data were used. RESULTS: More than 90% of SJS and TEN cases occurred in the first 63 days of AED use. Over the 4 years, increases in dispensing were 5% for CBZ, 65% for LTG, 6% for PHB, -16% for PHT, and 26% for VPA. Across a range of assumptions about frequency of incident use, the risk estimates vary between 1 and 10 per 10,000 new users for CBZ, LTG, PHT, and PHY and were consistently lower for VPA. CONCLUSION: Across a range of assumptions used, the risk of hospitalization for Stevens-Johnson syndrome or toxic epidermal necrolysis in new users is low for carbamazepine, lamotrigine, phenytoin, phenobarbital, and valproic acid. Because conservative incidence use fractions were used, it is likely that some risks were overestimated.
(32614) 2008 - Stevens-Johnson syndrome and toxic epidermal necrolysis: assessment of medication risks with emphasis on recently marketed drugs. The EuroSCAR-study.
J Invest Dermatol. 2008;128(1):35-44
Mockenhaupt M, Viboud C, Dunant A, et al
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but severe cutaneous adverse reactions (SCAR) related to a variety of medications. They have a significant public health impact because of high mortality and morbidity. A multinational case-control study conducted in Europe between 1997 and 2001 evaluated the risk of medications to induce SCAR. Cases were actively detected through a hospital network covering more than 100 million inhabitants. Three hospitalized patients per case matched on age, gender, and date of interview were enrolled as controls. After validation by an expert committee blinded to exposures, 379 SCAR cases and 1,505 controls were included. Among drugs recently introduced into the market, strong associations were documented for nevirapine (relative risk (RR)>22) and lamotrigine (RR>14), and weaker associations for sertraline (RR=11 [2.7-46]), pantoprazole (RR=18 [3.9-85]), and tramadol (RR=20 [4.4-93]). Strong associations were confirmed for anti-infective sulfonamides, allopurinol, carbamazapine, phenobarbital, phenytoin, and oxicam-NSAIDs , with some changes in relative numbers of exposed cases. Thus, many cases were still related to a few "old" drugs with a known high risk. Risk was restricted to the first few weeks of drug intake. The use of such drugs as first-line therapies should be considered carefully, especially when safer alternative treatments exist. A number of widely used drugs did not show any risk for SJS and TEN.
(33284) 2009 - Genetic basis for idiosyncratic reactions to antiepileptic drugs.
Curr Opin Neurol. 2009;22(2):144-149.
Franciotta D, Kwan P, Perucca E.
PURPOSE OF REVIEW: In recent years, there has been an explosion of genetic research in epilepsy, including a search for genetic markers of adverse reactions to antiepileptic drugs. This article will focus on recent findings concerning genetic factors affecting susceptibility to idiosyncratic reactions to antiepileptic drugs. RECENT FINDINGS: Recent studies have investigated the role of genetic factors in the development of antiepileptic drug-induced cutaneous reactions, carbamazepine and valproate-induced liver toxicity, vigabatrin-induced visual field defects, and antiepileptic drug-induced teratogenicity. The greatest progress has been an improved definition of the role of human leukocyte antigen-related genes as predictors of the risk of serious antiepileptic drug-induced cutaneous reactions. This has led to the recommendation that patients of Asian ancestry be tested for the HLA-B*1502 allele, in order to identify those at high risk of developing Stevens-Johnson syndrome and toxic epidermal necrolysis after administration of carbamazepine and, possibly, phenytoin and other antiepileptic drugs. SUMMARY: Future research will probably lead to discovery of additional genetic predictors of susceptibility to adverse reactions to antiepileptic drugs. Identification of genetic markers should, in turn, allow unravelling of the molecular mechanisms underlying these reactions. Ultimately, these advances should lead not only to improved personalization of therapy but also to development of safer drugs.

The Epilepsy Resource Center thanks you for sharing with the community and we look forward to hearing from you in the near future.

 

Cordially,

Gigi Jones

Information Specialist

EF's Epilepsy Resource Center

Thank you for your responses and information.  I see my neuro in a week.  He is an awesome dr.  We may stop the lamictal.

My SJS took about 8 weeks to resolve.

My cousin had it once as well. His actually started from cold sores on his lips (ie herpes) and got worse. I know about it. I do know lamictal can cause that rash...That is all I know..

How do these DRS NOT know about it?

Melissa I almost thought I was reading my sons medical history when you posted at first. Matthew has the same things happen and no doctor can explain it or knows of it. Matthew gets sick allot and lots of time they pump him full of heave antibiotics and this killing him. We spend days in the hospital. last time was 6 days and they could never tell me what was wrong with him. ulcers in the mouth, ulcers on his butt, he still has scars under his arm in a ring like pattern from last time. We have seen almost every specialist at Childrens as well. Its really sad. thanks for posting.

I am sorry your son is suffering.  What I have gone through is so painful.  I know I have had minor breakouts.  Please let me know what you find out and don't give up with a diagnosis.  Melissa B.

Melissa said:

Melissa I almost thought I was reading my sons medical history when you posted at first. Matthew has the same things happen and no doctor can explain it or knows of it. Matthew gets sick allot and lots of time they pump him full of heave antibiotics and this killing him. We spend days in the hospital. last time was 6 days and they could never tell me what was wrong with him. ulcers in the mouth, ulcers on his butt, he still has scars under his arm in a ring like pattern from last time. We have seen almost every specialist at Childrens as well. Its really sad. thanks for posting.

Sounds like you were on lamictal/lamotrigine. I'm so sorry this diagnosis of SJS has befallen you. All I can say is I hope you have caught it early and to wish you the best. I know this is a tad late, but I hope you have made it through with minimal damage.

Whoever told you they never heard of this are liars,Epilepsy medication can cause this such as Dilantin/Mysoline/depokate/Lamictal ,you can take these medication as I do for the past 40 years then boom get the SJS out of no where..

If your neurologist told you they never heard of it FIRE them I would that's my opinion.

Th following link is for education purpose only talk with your doctor
http://epilepsytalk.com/2009/10/17/stevens-johnson-syndrome-a-dange...
Melissa, if no one heard of "STEVEN JOHNSON SYNDROME" before they're idiot hate to say this but true.

What type of medication names you're taking with your seizures?

Here's some information on SJS
http://www.webmd.com/allergies/stevens-johnson-syndrome-11013V
http://www.mayoclinic.com/health/stevens-johnson-syndrome/DS00940
http://www.cnn.com/HEALTH/library/stevens-johnson-syndrome/DS00940....
http://www.hopkinsmedicine.org/wilmer/conditions/stevens-johnson.html


If I had this hope to god I don't get it,I wouldn't be typing on forums I would demand something be done..

Please educate yourself on this what the treatment is what can and can't be done etc,

Thanks for the info.  I have not had any symptoms of SJS in over a year maybe 2.  I am no longer taking lamictal which was the culprit.  I have not had to take any antibiotics.  I hope I don't either.  I am on a new AED, Onfi.  It is helping greatly with my seizures and my neuros at Duke are wonderful. 
 
CGG said:

Melissa, if no one heard of "STEVEN JOHNSON SYNDROME" before they're idiot hate to say this but true.

What type of medication names you're taking with your seizures?

Here's some information on SJS
http://www.webmd.com/allergies/stevens-johnson-syndrome-11013V
http://www.mayoclinic.com/health/stevens-johnson-syndrome/DS00940
http://www.cnn.com/HEALTH/library/stevens-johnson-syndrome/DS00940....
http://www.hopkinsmedicine.org/wilmer/conditions/stevens-johnson.html


If I had this hope to god I don't get it,I wouldn't be typing on forums I would demand something be done..

Please educate yourself on this what the treatment is what can and can't be done etc,

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