Steven Johnson Syndrome

Are you suffering from painful skin lesions? Do you also have blisters on your skin, mouth and anus? Is it accompanied by rashes all over your throat and face? You may be having Steven Johnson syndrome. Read more to know all about this disease, its causes, symptoms, effects and treatment.

What is Steven Johnson Syndrome?

Stevenson Johnson Syndrome is a serious systemic high magnitude allergic disorder characterized by an expression of Erythema Multifome – a rash on the skin and mucous membrane, which leads to extreme morbidity, and even death in extreme cases.

Picture 1 – Steven Johnson Syndrome
Source – ucsf.edu

This disease occurs as a result of allergic reaction to immunologic stimuli such as drugs and infectious agents. Apart from allergies, there may be other effects in the visual, vaginal, urethral, nasal and lower respiratory tract. This disease is Idiopathic (the cause is not known). It is a hypersensitive complex syndrome.

Steven Johnson Syndrome Symptoms

The symptoms of this disease vary in form and reaction from individual to individual. There are four major ways through which Stevens Johnson Syndrome manifests – eruptions in the skin, eruptions in the mucous membrane, peeling of skin, eruptions of both skin and mucous membrane. In the TEN (Toxic Epidermal Necrolysis) form of this disease, large areas of epidermis peel off.

The eyes and the mouth are seriously hampered when the effect is on the mucous membrane. The conjunctiva of the eyes is severely harmed.  The eye involvement can, at times, be permanent and may result in impairment of the vision.

Some of the common symptoms of this disease are:

• Itching and painful skin lesions
• Target shaped lesions in a bulls-eye pattern
• Skin blisters
• Mucous membrane lesions, blisters, swelling
• Rashes on the throat and blisters
• Mouth rash and blisters
• Genital rash
• Genital blisters
• Conjuctival rash
• Conjuctival blisters
• Tiredness
• Bloodshot eyes
• Skin conditions on arms, legs etc
• Swollen eyes, blisters may accompany
• Corneal blisters, holes and erosion
• Joint pain
• Cough
• Sore throat
• Malaise, feeling of illness, exhaustion
• Flu-like symptoms
• Vision loss
• Headache and Fever
• Mild Itching
• Mucous Membrane Ulcers
• Burning sensation
• Eye pain

Steven Johnson Syndrome Causes

Steven Johnson Syndrome arises primarily from a disorder of the immune system. Some of the possible medical causes of this disorder are as follows:

• Barbiturates
• Diltiazem
• Histoplasmosis
• Herpes virus 2
• Leflunomide
• Carbamazepine
• Stasphylococcal scalded skin syndrome
• Isoniazid
• Primidone
• Terbinafine
• Clindamycin
• Systemic Lupus Erythematosus
• Phenytoin
• Thiabendazole
• Orf
• Suramin
• Carbamazepine
• Procainamide
• Chlormezanone
• Ibritumomab tiuxten
• Staphylococcus aurus
• Penicillamine
• Ethotoin
• Mycoplasma Pneumoniae
• Nevirapine
• Lamatrogine

The consumption of some of the medications  also cause the Steven Johnson Syndrome.

Steven Johnson Syndrome and Bactrim

Bactrim is a brand name for an antibiotic that is used in combined antibiotic treatment. But it has some very deadly effects too; the most fatal is that it causes increased risk of causing the Steven Johnson Syndrome. In the initial stages of consumption of this drug, the patient may not show signs of this disease. But gradually, after two or three weeks, the symptoms start appearing clearly.

Steven Johnson Syndrome and Motrin

Motrin is a brand name for a non-steroidal anti-inflammatory painkiller that relieves you against a variety of ailments such as pains, feverishness and other similar aches. But recently, there have been concerns raised on the use of Motrin and the occurrence of the Steven Johnson Syndrome. This drug causes a number of side effects, including Steven Johnson Syndrome and blindness. Even the manufacturers of Motrin are seriously battling out this litigation.

The users of Motrin are often recommended to consult a lawyer. They may claim compensation, depending on the amount of damage caused. The law firm of the Thomas, proctor and P.A. is nationally recognized.

Steven Johnson Syndrome Bextra

Bextra is a drug prescribed for curing osteoarthritis, menstrual cramps and other adult pain. It belongs to the class of drugs collectively known as the cox-2 inhibitors. Though its benefits were many, however it had some severe side-effects, including Steven Johnson syndrome. The warning may come too late, as most people understand this after suffering from this disease. If you are taking Bextra, and you notice even the minutest signs of the Steven Johnson syndrome, consult a doctor immediately. You can claim compensation if there are any side effects and even legal help is available.

Steven Johnson Syndrome Lawsuits

Steven Johnson Syndrome is a life threatening disease and several lawsuits have been filed against the drugs which have caused this disease on consumption.

Steven Johnson Syndrome Stages

The stages of the Steven Johnson syndrome vary from mild to severe.  The early stage symptoms of this disorder are more or less identical to the common infectious and respiratory diseases, including malaise, cough etc. with the gradual development of the disease, lesions start developing throughout the body.  In the advanced cases, it leads to blindness. It may also cause scarring of the lungs in severe stages.

Steven Johnson Syndrome Complications

Apart from death, Steven Johnson syndrome may include some serious complications. These complications may involve the skin, or any other organ. The possible complications are:

Cellulitis – It is a secondary skin infection, hat produces life threatening complications such as meningitis (infection of the membranes and the fluid surrounding the brain and the spinal cord).

Eye Damage – Dry eyes, rashes or some other eye problem in one or more severe situation may lead to temporary or permanent damage to the eye tissue, inflammation, scarring or complete loss of vision.

Sepsis – It is a condition in which bacteria enters your bloodstream and spreads throughout the body. It can lead to complications such as shock and organ failure.

Skin Damage – A red to purple rash develops on the skin and the mucous membrane, sometimes in the shape of a target. Scars may remain; lasting skin problems may include peeling of skin into sheets, hair fall out, and abnormal growth of toenails and finger nails.

Internal Organ damage – Steven Johnson syndrome can cause inflammation and damage to the internal organs such as the lungs, heart, and the kidney. It causes Pneumonitis to the lungs, Myocariditis in the kidney and Hepatitis in the liver.

Genitourinary Complications – In the genital areas it may cause complications such as renal failure, renal tubule necrosis, vaginal stenosis and penile scarring.

Other complications caused due to this disease are:

• Arthritis
• Chronic fatigue
• Damage and scarring to the oesophagus
• Asthma
• Deformity
• Respiratory failure
• Loss of body fluids
• Corneal ulceration

Is Steven Johnson Syndrome Contagious?

Stevenson Johnson Syndrome is not contagious. But there may be cases, where some of its symptoms are contagious. The person suffering from this disease often looks disfigured, which make the onlookers think that it is contagious. But it is not.

Some of the non-contagious symptoms of this disease are: Skin reactions including burning, blistering, peeling, rashes. Fever and like symptoms are also non contagious.

Steven Johnson Syndrome Rash

Picture 2 – Steven Johnson Syndrome Image
Source – beauty-cosmetic-guide.com

Rashes are very common condition and may have many causes. Steven Johnson syndrome is characterized by rashes over the entire body. Rashes not only affect the skin, but they have their effect on the mucous membrane too. Rashes are accompanied by blisters on top.

Steven Johnson Syndrome Toxic Epidermal Necrolysis

Toxic Epidermal Necrolysis (TEN) is an acute, life threatening dermatologic emergency, induced by reaction to medications. Mycoplasma infections may also induce some infections. Steven Johnson Syndrome and Toxic Epidermal Necrolysis represent a spectrum of a single disease process. TEN can also be considered a very severe form of Steven Johnson Syndrome. Ten also causes the detachment of the top layer of the skin from the lower layers of the skin (dermis) all over the body.

Steven Johnson Syndrome vs Erythema Multiforme

Erythema Multiforme is a hypersensitivity reaction, characterized by targetoid lesions, skin eruptions, with or without blisters, in a symmetric distribution. It is mostly triggered by infections, chiefly by the herpes simplex virus (HSV).

Erythema multiforme (EM) is regarded distinct from the Steven Johnson Syndrome. Earlier, the two were often confused as the same disorder.

Steven Johnson Syndrome Diagnosis

Steven Johnson syndrome can be medically diagnosed based on the medical history, physical examination or by simply studying the distinctly appearing symptoms and signs. Usually a dermatologist is the clinician who should be referred to, in these cases. He may diagnose the disease with or without performing medical Biopsy. Some cases require the consultation of a surgery specialist, a pediatric consultant or a burn specialist.

Skin biopsy, a helpful laboratory study, involves examining the tissue sample of the skin under a microscope. Serum levels of the tumor necrosis are typically elevated in patients with this syndrome, but still the serologic test is not regularly used in diagnosing the Steven Johnson Syndrome.

Steven Johnson Syndrome Treatment

Since Steven Johnson syndrome constitutes a dermatological emergency, a dermatologist should be treating this disease. Those medications, which are known to cause any sort of allergic reactions, should be particularly discontinued. A precipitating agent should be detected and should be removed.

The initial treatment is similar to those of t6hermal burns. Care should be given, which must be both supportive – e.g. parental feeding and intravenous fluids; and symptomatic – e.g. analgesic mouth rinse. There is no other treatment beyond this. Corticosteroids are not generally recommended for treatment.

Often, fluid replacement is required to prevent infection. Some other agents, including Cyclosporine and Cyclophosphamide also have been used; although they have not reported therapeutic success. Sometimes, Intravenous Immunoglobin (IVIG) treatment is made use of, and it has reported success. The patient should immediately consult an ophthalmologist, as soon as The syndrome leads to the formation of scar tissue in the eyelids and causes impaired vision, corneal vascularization and other related eye problems.

Proper and effective treatment on the right time not only cures the prevailing problems, but it also prevents other problems from cropping up. At the end of medical treatment, a good physical therapy program will be helpful in making the patient more relaxed and comfortable.

Steven Johnson syndrome Drugs

Several medications are used to treat Steven Johnson syndrome. However, you should never consume any of the drugs at your own will. Always seek professional advice before you take any medication.

Some of the commonly recommended Steven Johnson Syndrome Medications are:

• Dexamethasone
• Decaderm
• Deenar
• Dex-4
• Decaspray
• Mymethasone
• Hexadrol
• Oradexon
• Turbinaire
• Solurex
• Neodexair
• Ocu-Trol
• Sofracort
• Maxidex
• PMS Dexamethasone

Steven Johnson Syndrome Prognosis

Steven Johnson syndrome is a rare condition, less than 10 percent of the total body surface is involved. It has a low mortality rate, which is only about 5 percent. Other outcomes are vision loss, cornea scratching, or organ damage. This risk can be estimated using a Scorten scale. It takes a number of prognostic indicators into account.

Steven Johnson Syndrome Pictures

You can have a look at these images to get an idea of the effects of this disease on the various parts of the body. You can see how a Steven Johnson Syndrome infected person looks like.

Picture 3 – Steven Johnson Syndrome Photo
Source – avimedi.net

Picture 4 – Image of Steven Johnson Syndrome
Source – avimedi.net

Steven Johnson syndrome is a severe, but a relatively curable disease. But it can lead to serious complications and effects on the skin and the body. Seek medical advice as early as possible. Early treatment will help you to get rid of this discomforting disease very soon.

Reference:

http://www.sjsyndrome.com/symptoms.html

http://en.wikipedia.org/wiki/Stevens%E2%80%93Johnson_syndrome

http://www.sjsupport.org/

http://emedicine.medscape.com/article/756523-overview

http://www.medicinenet.com/health_and_living/focus.htm

http://www.ehow.com/facts_5019632_stevensjohnson-syndrome-contagious.html

http://www.emedicinehealth.com/life-threatening_skin_rashes/article_em.html

http://www.skinassn.org/bactrim-septra-stevens-johnson-syndrome.html

http://www.drugs.com/sfx/bactrim-side-effects.html

http://en.wikipedia.org/wiki/Toxic_epidermal_necrolysis