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| What is Stevens-Johnson Syndrome & Toxic Epidermal Necrolysis? Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis are two forms of the same life-threatening skin disease that cause rash, skin peeling, and sores on the mucous membranes.
In Stevens-Johnson syndrome, a person has blistering of mucous membranes, typically in the mouth, eyes, and vagina, and patchy areas of rash. In toxic epidermal necrolysis, there is a similar blistering of mucous membranes, but in addition the entire top layer of the skin (the epidermis) peels off in sheets from large areas of the body. Both disorders can be life threatening.
Nearly all cases are caused by a reaction to a drug, most often sulfa antibiotics; barbiturates, or anticonvulsants. Some cases are caused by a bacterial infection: Occasionally, a cause cannot be identified. The disorder occurs in all age groups but is more common in people over age 40 people, probably because this age group tends to use more prescribed drugs. Stevens-Johnson syndrome and Toxic Epidermal Necrolysis usually begin with fever, headache, cough, and body aches. Then a flat red rash breaks out on the face and trunk, often spreading later to the rest of the body in an irregular pattern. The areas of rash enlarge and spread, often forming blisters in their center. The skin of the blisters is very loose and easy to rub off. In Stevens-Johnson syndrome, less than 10% of the body surface is affected. In Toxic Epidermal Necrolysis, large areas of skin peel off, often with just a gentle touch or pull. In many people with Toxic Epidermal Necrolysis, 30% or more of the body surface peels away. The affected areas of skin are painful, and the person feels very ill with chills and fever. In some people, the hair and nails fall out. The active stage of rash and skin loss can last between 1 day and 14 days.
In both disorders, blisters break out on the skin, mucous membranes lining the mouth, throat, anus, genitals, and eyes. What makes this condition so devistating is that the internal organs are blistered as well. The damage to the lining of the mouth makes eating difficult, and closing the mouth may be painful, so the person may drool. The eyes may become very painful, swell, and become so filled with pus that they seal shut. The corneas can become scarred. The opening through which urine passes (urethra) may also be affected, making urination difficult and painful. Sometimes the mucous membranes of the digestive and respiratory tracts are involved, resulting in diarrhea and difficulty breathing. New evidence points to horrible long-lasting repercussions that are coming to light as a result of this disease.
The skin loss in toxic epidermal necrolysis is similar to a severe burn and is equally life threatening. Huge amounts of fluids and salts can seep from the large, raw, damaged areas. A person who has this disorder is very susceptible to organ failure, as well as infection at the sites of damaged, exposed tissues. Such infections are the most common cause of death in people with this disorder.
For A Complete Understanding of This Life-Changing Condition, This Comprehensive and Detailed Book is the Best on the Market - and is only $12.99.
Stevens – Johnson Syndrome and Toxic Epidermal Necrolysis are insidious conditions in which the human body fools itself into thinking the skin and mucus membranes, both inside the body and outside the body, are foreign invaders; therefore the body attempts to rid itself of skin and internal organs. People who survive this horrible, life-changing condition face untold challenges on a daily basis. Until now, there has been no one published central informational reference about these conditions. If you are searching for answers on this condition, this book is for you.
 This unique book is the first of its kind, detailing all information known about this condition; describing the causes, signs and symptoms, and little known long-term effects. Includes treatment protocols for physicians, also includes a chapter written by Dr. Tseng on occular complications caused by SJS and TEN. This book contains invaluable information that may save your life, the life of a loved one, and it may help the survivor come to terms in living with this condition by knowing what to expect. Written by a survivor of Toxic Epidermal Necrolysis who is also a nurse.
My Experience with Toxic Epidermal Necrolysis
(The only reason that I'm writing this and placing it in public is because so little is known about this affliction. I thoroughly searched all the databases and the medical sites I could locate for answers and found very little. Being a premed student gave me access to medical school university databases that the general public does not have - and I still found very little. It is my hope that someone afflicted with SJS or TEN will find this and feel like you are not alone, and use this as a resource for information.)
In October 2006, I was taken acutely and violently ill. It came on quickly like the flu: Within hours; a gradually increasing sore throat, chills; nausea and vomiting with the inability to hold anything down, and a gradually increasing and unrelenting rising body temperature. I then noticed a rash that started on the trunk of my body. At first, the rash was pink and localized to my chest – within hours it turned into a blistering deep red rash that covered 70+% of my body. I sustained an oral body temperature of over 105F which antipyretics {Tylenol, and Advil} could not bring down. I was taken to the Emergency Room, where the resident ED physician treated me with IV antibiotics for what he believed to be a systemic blood infection, as I was already being treated with the Sulfa drug Bactrim DS for an active staph infection in my thigh. What lay ahead for me was straight out of a horror movie; changing life as I knew it forever.
I was admitted to a private room at the hospital. This condition is so rare that various hospital physicians were brought in to see me that had never encountered this disease before. I grew progressively worse as the unrelenting fever, chills, nausea and vomiting continued. The deep red rash slowly grew into raised blisters both small and large that filled with fluid engulfing most of my body, including the palms of both hands and soles of both feet. An Infectious Disease Physician was called in to evaluate me. After extensive questioning and conferring with other physicians it was narrowed down to two conditions: A disease that is native to the Congo region of Africa, and Stevens - Johnson syndrome. According to the attending physician, these are the only two known conditions that will cause a rash to manifest on the palms of hands and soles of feet. The blisters continued to develop and detach the first layer of skin from my body: I became like a burn patient that day. Blisters developed on 70% of my body; the largest one blister that I remember covered most of my chest area, and was almost 2-inches deep with fluid. The needle of the intravenous line that dripped ringers lactate / sterile normal saline into my arm kept slipping out because fluid filled blisters covered both my arms, so they had to keep restarting the IV – and taping it to stay in. No one knew; not even me with all of my medical knowledge and experience, that when the tape was removed, so would be the first layer of skin of most of my right forearm. The pain is indescribable when one is literally filleted like a fish. The scarring of that act, both mentally and physically, will be with me all of my life.
For three days the fever continued to rage and the rash continued to turn into fluid-filled blisters. At one point a physician informed me that I was scheduled to have my body packed in ice in an attempt to bring down my body temperature. The nursing staff at the hospital had no experience with this malady and there was no set nursing protocol to abide by. By this time, blisters had filled the entire area of my mouth and throat. Both my lips looked as if they would split open at any moment engulfed by what seemed like one lone cold sore that melded into smaller blisters on my face. My gums were blistered. My eyelids were yellow fluid filled sacks of skin that wept exudates continuously. Every movement of my body caused the smaller blisters to burst open and weep. When the fluid from the blisters dried, it formed scabs which in turn tore open with body movements. It seemed that every part of my body was affected: I couldn’t move my fingers without blisters bursting open forming scabs that in turn opened too. No one realized that what was happening on the outside of my body was also happening on the inside of my body as well. They packed me in ice to bring down my fever, and then placed me on “reverse isolation” and sterile precautions. Blisters were both on the inside, and the outside of my body. Blisters covered my throat and breathing passages. Finally I was given Lidocane “swish” for my blistered mouth, gums, and tongue so that I could stand to take small sips of water again.
On day three in the hospital, I was transferred via EMS to another hospital better equipped to handle patients in my condition. I remained on reverse isolation, and sterile sheets were used on my bedding. Everyone who entered the hospital room was required to wear a mask and gown for my protection. Also in the second hospital, a PICC line was placed into my right arm to alleviate the use of IV lines. [A PICC line is an IV line that is placed deep inside the arm that runs from the heart to the outside of the skin so as to alleviate reinsertion of a standard intravenous line needle.] The medication regime included Lidocane swish, IV steroids, and insulin shots. Precautions included sterile sheets and bedding, a daily room cleaning with a special solvent, masks and gowns for each person that entered the room, and daily showers using a Betadine solution to kill germs. I went from a liquid diet to a pureed diet to a soft food diet during my 12-day hospital stay. The blisters burst and formed scabs – scabs that broke open and bled for the next 3-weeks. It was two-months before I could brush my teeth again because of the blisters on my gums.
I’m told I’m lucky to be alive.
Fast-forward four months: I've been tested for every kind of virus known to man it seems; lucky for me all tests are negative. Life is slowly returning to normal. I remain under the care of an Internal Medicine Specialist. Chronic fatigue has become a bothersome neighbor. Blisters remain intermittently on the inside of my lips. One scar from a blister has found a home on my bottom lip. Blisters remain on my eye-lids. Blisters come and go inside my nasal passages and sinuses. I have suffered difficulties because of the blistering of the inside of my intestines. The rash will come and go on my body – no one knows what brings it on. My blood work is all screwed up and I am being watched from everything from Lupus to Leukemia because of this affliction. I am being watched for Basil Cell Carcinoma on my lip because of TEN. My immune system is compromised. I am photosensitive. I carry physical scars from the blisters on my arms, chest, and face. My fingernails and my toenails literally lifted off their beds because the underlying skin blistered – my toenails refuse to fully grow back. On the palms of my hands and the bottom of my feet – the skin became one large fluid-filled blister and it lifted off in one large sheet so the skin is still fresh there. I have fresh skin on most of my body that I must be acutely aware of. I have now returned to work and I am starting to accept the fact that there is nothing I can do but take things as they come. I take medications as a result of TEN that stabilizes my body chemistry [Potassium and Acidophilus Probiotic], and Genteal gel for my eyes. I also use EVERYTHING that I bathe with or wash with that hs "no added dye or colorings".
Present day: I'm almost back to my old self again. I will always have the scars from the blisters, but I'm learning to live with that fact. A scar from a blister still inhabits my lip and I'm a bit self-conscience of it. My fingernails have grown back; the "quick" of my toenails has finally come back. Frequent reoccurrences of lesser manifestations of SJS come and go: The manifestations are just part of my life now and I've accepted things as they are. I still see a physician at least once a month and get blood-work done; they still watch for lupus, leukemia, and a few other things that may have been triggered by having all the fluid sucked out of my internal organs during my initial manifestation several months ago. My eyes and lips still give me problems. I can't say that my body has returned to 100% homeostasis yet, but it's trying. Chronic fatigue is still my neighbor. One indomitable key to this affliction is acceptance.
I have published a book on SJS – please look for it at Lulu Press on Lulu.com by using the above link. If you have experience with SJS or TEN, please share your experience with the world. So little is known about this deadly affliction that by sharing your experience, you will help survivors to cope with this affliction. Please use the "Contact Me" tab above to write and include your story with SJS or TEN. Please feel free to write me if you or someone close to you is experiencing SJS or TEN and want to ask questions, or if you simply want to say "hello".
EM/ SJS/ TEN Facts History: Stevens - Johnson syndrome was first described in 1922. Stevens-Johnson syndrome (SJS) is an immune-complex–mediated hypersensitivity complex that is considered a severe expression of erythema multiforme. The progression shows the severity variant on a unilateral diagnosis line as follows:
Erythema Multiforme → Stevens – Johnson Syndrome → Toxic Epidermal Necrolysis
Erythema multiforme (EM) was initially described in 1866 by Ferdinand von Hebra as an acute self-limited skin disease, symmetrically distributed on the extremities with typical and often recurrent concentric "target" lesions.
Stevens-Johnson syndrome (SJS) was considered an extreme variant of EM for many years, while toxic epidermal necrolysis (TEN) was considered a different entity. However, in 1993, a group of medical experts proposed a consensus definition and classification of EM, SJS, and TEN based on a photographic atlas and extent of body surface area involvement. According to the consensus definition, SJS was separated from the EM spectrum and added to TEN. Essentially SJS and TEN are considered severity variants of a single entity. The two spectra are now divided into (1) EM consisting of erythema minor and major (EMM) and (2) SJS/TEN.
Pathophysiology:
EM / SJS is an immune-complex–mediated hypersensitivity disorder that may be caused by many drugs, viral infections, and malignancies.
Causes: Approximately 50% of cases are idiopathic, with no precipitating factor identified. Many potential biological and chemical triggers have been implicated as possible causes of EM, SJS, and TEN. Most notably causes are infectious agents and drugs. All 3 disorders are linked to drugs with TEN being exclusively attributed to this factor. Infectious causes are more common in children and are implicated more commonly in EM. Herpes simplex infection {Either HSVI or HSVII} is the most common cause in young adults and is strongly associated with recurrent EM. The most prevalent bacterial precipitant is Mycoplasma pneumoniae.
Signs & Symptoms:
Erythema multiforme:
Sudden onset of rapidly progressive, symmetrical, and cutaneous and/or mucocutaneous lesions, with concentric color changes in some or all lesions
Centripetal spread
Burning sensation in affected areas
Pruritus generally absent
Nonspecific prodromal symptoms suggestive of a viral syndrome in at least 50% of cases, usually 1-14 days before skin lesions develop. Symptoms may include fever, malaise, myalgias, arthralgias, headache, sore throat, cough, nausea, vomiting, and diarrhea.
SJS/TEN:
Generalized cutaneous and/or mucocutaneous lesions with blisters
May include symptoms of fever, malaise, myalgias, arthralgias, headache, sore throat, cough, nausea, vomiting, and diarrhea
Oral pain, which may be severe enough to result in difficulty eating, drinking, or opening the mouth
Eye pain, edema, and drainage
Breathing difficulty resulting from tracheobronchial involvement
Dysuria
What to expect:
Erythema multiforme:
Symmetrically distributed, erythematous, expanding macules or papules evolve into classic iris or target lesions, with bright red borders and central petechiae, vesicles, or purpura.
Lesions may coalesce and become generalized.
Vesiculobullous lesions develop within preexisting macules, papules, or wheals.
Rash favors palms and soles, dorsum of the hands, and extensor surfaces of extremities and face.
Postinflammatory hyperpigmentation or hypopigmentation may occur.
Eye involvement occurs in 10% of EM cases, mostly bilateral purulent conjunctivitis with increased lacrimation.
Mucous membrane blistering occurs in about 25% of cases of EM, is usually mild, and typically involves the oral cavity.
SJS/TEN:
Fever is common.
Skin findings may be similar to EM but often are more variable and severe. Inflammatory vesiculobullous lesions, often with hemorrhage and necrosis, are typical. Fixed macules and target lesions may be larger and more confluent than in EM.
Facial edema or central facial involvement
Mucous membranes are strongly affected, most commonly mouth, lips, and bulbar conjunctivae; less often, anogenital mucosae are affected. Lips may be edematous, bloody, or crusted. A minimum of 2 mucosal surfaces must be involved; 3 mucosal surfaces are involved in about 40% of cases.
Blisters or epidermal detachment less than 10% BSA for SJS and more than 30% for TEN; the outer layer of the epidermis separates readily from the basal layer with lateral pressure (positive Nikolsky sign).
Bullae and shallow ulcers resembling aphthous ulcers are common. When bullae rupture, mucosal lesions become deeply erythematous erosions, often covered by gray pseudomembranous exudates.
Salivation often is increased.
Nasopharynx, respiratory tract, GI tract, and genitourinary (GU) tract are sometimes affected.
Genital involvement consists of hemorrhagic, bullous inflammation; urinary retention and phimosis may occur.
Eye involvement occurs in approximately 85% of cases. These range from hyperemia to extensive pseudomembrane formation. Synechiae between eyelid and conjunctiva often occurs. Keratitis and corneal erosions are less frequent.
Treatment: Because this condition is so rare, the medical community seems to agree on "generalities" rather than one set-in-stone treatment plan for SJS / TEN.
People with Stevens-Johnson syndrome or toxic epidermal necrolysis are hospitalized. Any drugs suspected of causing the disorder are immediately discontinued. When possible, people are treated in a burn unit and given scrupulous care to avoid infection. If the person survives, the skin grows back on its own, and unlike burns, skin grafts are not needed. Fluids and salts, which are lost through the damaged skin, are replaced intravenously.
Use of corticosteroids to treat the disorder is controversial. Some doctors believe that giving large doses within the first few days is beneficial, whereas others believe that corticosteroids should not be used. These drugs suppress the immune system, which increases the potential for serious infection. If infection develops, doctors give antibiotics immediately.
In many cases, doctors give intravenous human immunoglobulin (IVIg) to treat toxic epidermal necrolysis. This substance helps to prevent further immune damage to the skin and further progression of blistering.
Toxic Epidermal Necrolysis, depending on the involvement of skin, has a very high mortality rate.
{www.EMedicine.Com, Merck.Com, W. A. Boyer, et tal}
The above treatment is the “accepted norm” in the medical community at large. Since this condition is so rare, it is my learned opinion that experiences and treatments differ to a degree between victims and practitioners.
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