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Thursday, February 26, 2009

erythema multiforme/Stevens-Johnson syndrome

I took Madison to the Doctor today. She has erythema multiforme/Stevens-Johnson syndrome. They said they would have wanted to see her yesterday if they new it was as bad as it is, but it was worse this morning so I guess it was best I took her today. She looks really bad I am really scared for her. She was seen by three different Doctors and all were very concerned. The horrible thing for her is that there is no treatment unless it stages get worst she is at about a 6 right now, 10 being death! So I am a little freaked out.

Here is a little about erythema multiforme/Stevens-Johnson

Erythema multiforme is a type of allergic reaction that occurs in response to medications, infections, or illness. Medications include:

  • Barbiturates
  • Penicillins
  • Phenytoin
  • Sulfonamides
Erythema multiforme (also known as Stevens-Johnson syndrome [SJS]) and toxic epidermal necrolysis (TEN) are often confused in the medical literature.

In 1860, Ferdinand von Hebra initially described erythema multiforme as an acute, self-limited condition with characteristic red papular skin lesions.1 The papules evolve into pathognomonic target lesions or iris lesions that appear within a 72-hour period and begin on the extremities. Lesions remain in a fixed location for at least 7 days and then begin to heal. Precipitating factors include herpes simplex virus (HSV), Epstein-Barr virus, and histoplasmosis. Because this condition may be related to a persistent antigenic stimulus, recurrence is the rule rather than the exception, with most affected individuals experiencing 1-2 recurrences per year. Erythema multiforme is typically a benign, self-limited disorder.

SJS is a mucocutaneous disorder. It was first described by Stevens and Johnson in 1922 as febrile erosive stomatitis, severe conjunctivitis, and disseminated cutaneous eruption.2 Lesions typically begin on the face and trunk. They are flat, atypical lesions, described as irregular purpuric macules with occasional blistering. Most patients also have extensive mucosal involvement. More than 50% of all cases are attributed to medications. This is a more serious illness and is potentially life threatening.

The confusion between these two separate clinical entities began in 1950, when Thomas coined the terms erythema multiforme minor and erythema multiforme major to describe conditions he encountered.

  • Erythema multiforme minor was applied to patients with the illness originally described by von Hebra as erythema multiforme.
  • Erythema multiforme major was applied to patients who also displayed oral mucosal involvement, similar to that described by Stevens and Johnson.

Up to 50% of patients with HSV-associated erythema multiforme have been found to have oral ulcers. However, this is now recognized as a variant of erythema multiforme, rather than SJS. Because SJS and erythema multiforme have different precipitating factors and different clinical patterns, the terms erythema multiforme major and erythema multiforme minor should no longer be used.

  • Erythema multiforme with mucosal involvement is now termed bullous erythema multiforme.
  • SJS is recognized as a separate clinical entity.

Lyell first described TEN in 1956.3 His original description made no reference to the work of Stevens and Johnson. The distinction between SJS and TEN is not clear. In fact, these conditions probably represent differing severities of the same disease process.

SJS and TEN have similar precipitating factors, identical histopathologic lesions, and similar clinical patterns. By current convention, the following terminology is used:

  • The term SJS is used when the disease involves less than 10% of the total body surface area.
  • TEN is used when the disease involves more than 30% of the body surface area.
  • Patients whose disease involves 10-30% of their body surface area are said to have SJS/TEN overlap. Mortality increases as the percentage of involved body surface increases, making TEN the more severe of the skin reactions.

I just ask that all of you pray for her. Please ask the Lord to watch over her and help her heal fast. I trust in the Lord with all my heart and know that in times like these I am so greatful he is in my life. I truly don't know what I would do with out the Faith I have in him.

I opened up my bible and just read and these stood out to me I thought they just fit.

"Therefore do not worry about tomorrow, for tomorrow will worry about itself. Each day has enough trouble of its own." Mt 6:34

"Come to me all who are weary and burdened and I will give you rest." Mt. 11:28

Thank you all for thinking about Madison and Praying for her. I will keep you updated on her condition as it gets better or worse.


~Cyn~

27 comments:

Courtney said...

Oh no! Poor baby. I'll say a prayer for her right now.

Cynthia said...

Thanks so much Courtney!

Susanne said...

Poor little Madison! She is in my prayers. ♥

Anonymous said...

I have the EM thing myself but for many its a one off
I pray thet the little one gets back to how God wants her to be and she knows his heaing touch.

John Newcastle England

Jeremy and Cindy Hunt said...

I was recently told that my eleven month old has erythema multiforme after she had a reaction to amoxicillin. She had been taking it for nine days when she started breaking out in a rash. We thought it was something she ate at first. We gave her the last dose of her meds the next day and she broke out worst and had painful swelling of her hands and feet. It was terrible to see her crying when we touched her. The doctors haven't been very helpful in helping us understand what to expect or worry about so I appreciate your information and sharing what your experience has been like. We will just be glad when it is over.

erinspice said...

I'm sorry your daughter had to go through this. My 4 year old also develops EM when she is exposed to penicillins or the chemically similar cephalosporin antibiotics. She's had two episodes of EM, at 13 months (amoxicillin) and 3 years (ceftin). The second time was much easier to deal with because we knew we were giving her a potentially reactive drug (a small percentage of those allergic to penicillins are also allergic to cephalosporins), so we monitored her very closely for sign and caught it within a couple of hours. If you're interested, I have photos of her first episode here and her second episode here. Your daughter is almost certainly better by now, but I hope she never has to deal with EM again.

A.J. said...

Hello,

My Daughter has had SJS twice this past year. I am wondering what you do to avoid repeats. Do you avoid certan medications? Does your child medical alert? What where you told to do if it happens again. I am surpised by how clueless the doctors where. I insisted on a skin biopsy the second time. Even then they said it was EM Minor. I brought my daughter to the Mayo and they informed me that it was Major and SJS.

My daughter is 6 reactions where from amoxicillin and vaccines (DPT Hep A, I don't know which) She was being re-vaccinated due to receiving her baby shots out of the US.

erinspice said...

A. J. - We have pinpointed our daughter's allergic reactions to two classes of antibiotics, penicillins and cephalosporins, both of which contain many individual drugs. Ibuprofen is also a common cause of EM/SJS. Did you give your daughter ibuprofen before or after the vaccinations when she developed EM/SJS? Concerning the antibiotics, there is a small link between penicillin and cephalosporin allergies. The two drugs have similar shapes, so 5-10% of people who are allergic to penicillins will also react similarly to cephalosporins, like those in the list on Wikipedia's page about cephalosporins. Note that 3rd gen cephalosporins are not as similar, so some people who are allergic to most cephalosporins will not react to 3rd gen cephalosporins. We're lucky that Tru is one of those lucky few. She can take Omnicef without reacting.

If your child has EM, I would continue avoiding any form of penicillin, obviously including amoxicillin; watch closely the first time she takes Omnicef, and the first time she takes a non-3rd-gen cephalosporin like Ceftin; and avoid ibuprofen.

Our daughter does wear a Medic Alert bracelet. My reasoning is that if our family were involved in a car accident and she was injured, the first thing they'd do at the hospital is administer broad-spectrum antibiotics. If I or my husband is not alert enough to tell them she's allergic, it could be disastrous. Allergies can morph forms. An immune response allergy like EM/SJS could morph into an anaphylactic allergy as the child ages. It would really suck to rely on the fact that her reaction is non-life-threatening (like EM minor) only to have her develop an anaphylactic allergy.

Anonymous said...

Hi my name is David,
I am currently a nursing student doing a research paper on the SJS/TEN disease. All the papers and journals point to intense IVIG treatment. Some of the others also apply a corticosteroid treatment. I don't pretend to even know enough about it to do any type of diagnosing but it wouldn't hurt to ask the doctor about these treatments. The University of Florida medical journal has a 26 step guideline for dealing with this specific disease which covers using the IVIG treatment. I will be praying for her complete recovery. May God be with you and comfort you all through your days.

MOMMY of a SJS Survivor said...

I can honestly say I know EXACTLY how and what your feeling. Six days after my baby turned three she contracted SJS. She looked JUST LIKE your daughter. My daughter was NOT ON ANY MEDICATION at the time of her out break. I took her to the dentist for her first visit, she hopped up in the chair, thats when I seen the red dots on her arms. When we got home I called her doctor. I was told it was the chicken pox and to give her childrens motrin to keep her compfortable. Two ER vistis and a hospital admission later we were told that she had SJS. I thank GOD evewry day that my little babies skin did NOT slough off, she swelled up really bad. She now has to take speech cleasses, due to the blisters on the roof of her mounth, she can not tolerate the heat out side, she does not sweat. I am trying to spread awareness and to meet other parents like you and I, here in Ohio, I am being interview by our local news on Monday the 17th.

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