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What is Steven Johnson Syndrome?

What are the causes of Steven Johnson Syndrome? Do we have this syndrome in Nigeria? All the characteristics of this disease are already gathered here!

Steven Johnson Syndrome

Steven Johnson Syndrome is acute bullous lesion of the mucous membranes and skin. Lesions have allergic nature and usually occur on the background of the serious condition of the patient.

At the same time the pathological process may involve oral mucosa, urinary organs and eyes. The disease is accompanied by the formation of many bubbles on different areas of skin and mucous membranes.

The first information about this syndrome appeared in 1922. The name of this pathology was chosen in honor of the authors who first described it. Steven Johnson syndrome can appear at any age. It is most commonly develops between the ages of 20-40 years. Much more rarely it can occur in the first three years of life. Many experts say that men are ill more often than women.

Steven Johnson Syndrome causes

Steven Johnson Syndrome causes

The cause of Steven Johnson syndrome is usually immediate type of allergic reaction. In total there are four groups of factors that may cause the onset of the disease - it is drugs, infections, malignant and unspecified causes of disease.

In children’s bodies this syndrome often occurs because of some viral diseases. Such diseases include herpes, viral hepatitis, measles, adenovirus infection, influenza, mumps and chicken pox. This syndrome can be provoked both by bacterial and fungal infections. Examples of bacterial infection are tuberculosis, salmonellas, yersiniosis, gonorrhea, mycoplasmosis and brucellosis. Examples of fungal infections are histoplasmosis, coccidioidomycosis and trihofitia.

Steven Johnson Syndrome symptoms

In adults’ bodies the development of this syndrome is associated with taking certain medications or with certain malignant processes. Some drugs, which can cause the development of Steven Johnson syndrome, include antibiotics, nervous system regulators, steroidal anti-inflammatory drugs and sulfonamides. Among cancers, carcinoma and lymphoma play a major role in provoking the syndrome. If the cause of the disease cannot be found, then one speaks of idiopathic form of Steven Johnson syndrome.

Steven Johnson Syndrome symptoms

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Steven Johnson syndrome usually begins acutely and the pathology symptoms develop quite rapidly. The onset of the syndrome is generally similar to the development of infectious respiratory diseases. Notable skin rash appears after about four to seven days after illness onset. At the same time the symmetrical rash consisting of red swollen papules appears on the skin. The size of papule in diameter may be 2-5 centimeters. In the center of each vesicle formation it is filled with serous or hemorrhagic content. These bubbles are easily injured, so they can be easily opened. After opening the bubbles there appears the mucosal erosion bleeding and yellowish serous cover coating. The appeared rash is usually accompanied by itching and burning sensation. After about two weeks the patient has symptoms such as cough, fever and general weakness.

Steven Johnson syndrome on body

Let us consider the further development of the symptoms of the disease. So, at first a patient notes headache, malaise, severe weakness, body temperature, which rises to 40 degrees, there is tachycardia, muscle pain and tenderness of the joints. Also, patients complain of pain in the throat, vomiting, diarrhea and cough. After a few hours there appear quite large bubbles in the mouth. When they open, there appear extensive defects in the mouth; they are covered with yellowish or white-gray pellicle. Also crust of dried blood is formed on these defects. In addition to the oral mucosa in the pathological process the red border of lips are involved there too. Due to defects in the mucous membrane the patient meets difficulties not only eating, but even drinking. Most often Steven Johnson syndrome affects extensor surface of the forearm, wrist, the front side of the feet, legs, face, as well as the genitals.

Steven Johnson Syndrome on lips

If you have eye disease, at the beginning it usually looks like the allergic conjunctivitis, but it is often complicated by secondary infection with developed purulent inflammation. Steven Johnson syndrome also can form ulcers elements on the cornea and conjunctiva. Such elements typically have a small size. In addition, there is the probability of hitting the iris, as well as the development of keratitis, blepharitis and iridocyclitis.

In 50% of cases this syndrome affects mucosal lesion of the genitourinary system, which occurs in the form of vulvitis, urethritis, vaginitis and balanoposthitis. Because of the formation of erosion and ulcers urethral stricture may suddenly appear.

a woman scratching

The lesions of the skin with this syndrome usually look like ‘towering’ rounded purple elements up to three to five centimeters, similar to blisters. Usually there are a lot of such elements. Most often the rash appears on the trunk and in the crotch. New lesions appear in about 2-3 weeks. Ulcers heal only in 1.5 months. The complications may be: bleeding from the bladder, colitis, pneumonia, bronchiolitis, acute liver failure, vision loss, and secondary bacterial infection. Due to these complications 10% of patients with Steven Johnson syndrome die.

If a person has this syndrome he or she needs immediate assistance. Primarily, it is necessary to conduct peripheral vein or central vein catheterization. This is necessary for infusion therapy.

Steven Johnson Syndrome diagnostics

a severe cold

The diagnosis of the syndrome needs a thorough examination of the patient. In addition, immunological blood test is carried out, as well as coagulation and skin biopsy. As additional diagnostic methods doctors may use ultrasound of the kidneys and bladder, lung X-ray and biochemical analysis of urine.

During the diagnosis of Steven Johnson syndrome a dermatologist relies on characteristic symptoms that are easily detected during a thorough dermatological examination. During the examination of the patient a doctor can determine a causal factor for the development of the syndrome. Skin biopsy is used to confirm the diagnosis of Steven Johnson syndrome. As for the diagnosis the doctors also use histological examination. It reveals necrosis of epidermal cells, subepidermal blistering and perivascular infiltration of lymphocytes.

Steven Johnson Syndrome treatment

Steven Johnson Syndrome on back

The main methods of treatment of this syndrome are the extracorporeal hemocorrection, infusion and glucocorticoid therapy. As the treatment doctors also use antimicrobials. Furthermore, as the treatment of Steven Johnson syndrome it is common to use formulations with adrenocortical hormones. If the patient is in serious condition, then these drugs are injected intravenously. Drugs are generally used as long as the patient feels better. After that the doctor will gradually reduce the dose, and when there is complete recovery, injections of drugs are ended.

Besides hormones the treatment of disease sometimes requires using drugs that promote excretion of toxic substances. In some cases, there must be infusion of blood plasma and protein solutions. To treat the lesions of the mucous membranes and skin doctors use creams or use some other ‘solutions’, which contain the same adrenocortical hormones in their composition.

Steven Johnson syndrome and children

Steven Johnson syndrome and children

READ ALSO: Did wild polio virus return to Nigeria?

The causes of Steven Johnson syndrome in children’s bodies is considered to be using anti-bacterial drugs. These drugs include sulfonamides and anti-microbial agents. Also syndrome may be caused by non-steroidal anti-inflammatory agents. These include phenacetin and salicylates. Among the medicines, which are promotional for this syndrome, we also should note sedatives and barbiturates. The development of the syndrome can be triggered by inflammatory diseases, which have a viral origin. During such diseases the child's body is weakened and it leads to the increased sensitivity of the patient's organism to drugs.

At the beginning of the development of disease in the child's body temperature rises to 38-40 degrees, there is redness, pain in the throat, pain in the oral mucosa. In addition, there may be a nasal congestion, pain in the joints, and some children even have herpes of the lips, in the external genitals, as well as on the oral mucosa.

sick kid

Also bright purple rash forms on the skin of a child with this syndrome. Then on the place of the rash you may notice appearing bubbles with a thin cover. They are usually filled with serous-hemorrhagic content. After their opening the bladder erosion is formed there. Often the patients with Steven Johnson syndrome have the development of the bilateral conjunctivitis, which is further complicated by keratitis. Eruptions can stay up to two weeks. A lot of children also have genital lesion, such as vaginitis, urethritis, and balanoposthitis. Some children may also have liver inflammation, as well as the probability of occurrence of inflammatory changes in prianal area, changes in the lungs, the nervous system, heart and kidneys.

The laboratory studies of pediatric patients revealed anemia, increased white blood cells and increased level of ESR. During this syndrome usually there is increased body temperature for one or two weeks. Primarily conducting the diagnosis the doctors take into account data history and clinical examination. In some cases - the allergic history data and the results of allergodiagnostic methods in vitro.

Steven Johnson syndrome on skin

In the case of a favorable course of the syndrome as a result there is recovering, normal structure of the skin. On the place of erosion (after the epithelialization process) there may appear enhanced pigmentation of the skin. There is a possibility of recurrence of the syndrome, which usually lasts from a few months to a year.

If the doctors carry out adequate and timely treatment, the sick children usually recover. The life threat appears only if there are some severe complications of the clinical forms of the syndrome. During the treatment of Steven Johnson syndrome for children doctors use the same methods as for the treatment of toxic epidermal necrolysis. Furthermore, using disinfectants and binding solutions doctors treat oral mucosa, genitals. If there are lesions of the anterior eye segment, they use 30% solution Albucidum and 1% hydrocortisone suspension. Treatment in this case should be monitored by ophthalmologist.

Steven Johnson Syndrome. Situation in Nigeria

sick man

Professor Isaac Adewole, who is the Nigerian Minister of Health, said in opposition to different reports that Steven Johnson Syndrome or SJS is a rare disease caused by adverse drug reactions, it is not a new disease, and it was not detected in Nigeria.

Adewole also cautioned all Nigerians to avoid any self-medication and to be completely mindful of Steven Johnson Syndrome. He added that the disease first appeared in 1922 and could occur because of the use of some prescribed drugs.

Isaac Adewole

There appeared issuing warning statement called ‘Steven Johnson syndrome in Nigeria: Setting the record straight’. Nigerian Minister called on people to report about every case of any wrong drug reactions through Pharmacovigilance unit of our National Agency for Food and Drug Administration and Control, NAFDAC.

This year on the 13th of May Isaac Adewole flagged off a long-term 17-day marathon race from Abuja to Lagos, which was organized by the Federal Ministries of Health and also Information and Culture in tight partnership with a famous marathon runner, whose name is Mr Fadesola Adedayo. In 2012 his brother, Adeyosola Adedayo, unfortunately, died due to an unfavorable drug reaction to drugs he took after maintenance of a needle stick injury when he was managing a patient sich with HIV.

Nigerian doctor

 ‘He ran Teaching Hospital Idi-Araba for Steven Johnson Syndrome in the Lagos University, and he died only in a few days because of the severity of this disease’. Adewole praised the positive attitude of marathoners, remembering the death of his brother, creating awareness through their 17-day marathon said Steven Johnson syndrome is not a new sickness at all. Supporting the marathon of Mr. Adedayo, all the federal health agencies were directed to Adewole to offer free first aid and rescue services to the runners. Previously, Mr. Falodun, who is a consultant at the National Hospital in Abuja, gave a long lecture on the topic of Steven Johnson Syndrome, stressing that it is a rare disorder that can appear after drug use. He described all the general symptoms, different health complications and also treatment options.

Steven Johnson Syndrome pictures are very scary, but do not rush to find this disease in your body. It is very rare, but if you have some common symptoms, immediately visit a doctor!

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