By: Dr. Ameya Tripathi ( Special Correspondent-ICN Group )
LUCKNOW: These days we frequently hear about things like drug-resistant and genetically adapted forms of various disease causing microbes and bugs. Time and again we are alarmed by the extent to which people have developed the habit of pill-popping or self-prescription for all kinds of illnesses.
In India, especially, this is prevalent due to several factors like availability of medicines without prescription, immoral practice of quackery, lack of public awareness and so on. All this is just one facet of the overall catastrophe that self-prescription and ignorance can bring on us.
Majority of us would have never even heard of a dangerous syndrome that results from side effects of medicines apart from other causes. Just imagine a scenario: You are having fever or flu like symptoms, followed by red or purplish rash on your lips or skin. These rashes spread and blister. Gradually the skin starts to shed. What do you do? Just your usual: some analgesics, antibiotics…may be even some skin ointment. But wait. All these could just aggravate your symptoms! Because it can be SJS (Stevenson- Johnson’s syndrome)! It’s time to immediately visit your physician.
Now, what is this SJS? Well, at best Stevenson Johnson syndrome can be defined as a medical emergency which needs hospitalization and thorough treatment under qualified doctors as it is a limited form of toxic epidermal necrolysis (TEN).
This disorder affects the skin, mucous membranes and eyes. Stevens-Johnson syndrome occurs twice as often in men as women, and most cases appear in children and young adults under 30, although it can develop in people at any age. The disease affects skin and mucosa. Its starts with red or purple small patch which can exfoliate and skin regenerates on its own or many times can lead to complications.
The treatment is not only for the underlying cause but for controlling the spread, and for helping patients in the regenerative phase and avoiding complications during it.
Many a times the symptoms are mild and are overlooked if a blister occurs following flu. The skin above the blister dies, sheds off and eventually regrows. People in India tend to avoid doctors unless some emergency occurs, and this tendency can be dangerous in case of Stevenson Johnson syndrome.
Let’s take a look at the causes of this disease.
This is a rare, unpredictable reaction of skin and mucous membranes of body and the exact cause cannot be identified in many cases but it appears following an active infection or as a reaction from several medications. Stevens-Johnson syndrome is usually caused by an allergic reaction to a drug or medication.
The reaction from medication can happen while you are taking it or even up to 2 to 3 weeks after you have stopped it. A weakened immune system increases chances of Stevenson Johnson’s syndrome. As we know, the immune system gets weak as a result of chronic infections, autoimmune disorders, organ transplants or any other immune-compromising conditions. Family history of this disease is also a factor to consider. If any of your direct bloodline had this disease, there are high chances that u can develop this too.
A gene has also been identified as HLA-B 1502 GENE which predisposes people to SJS, particularly if you are taking medications for seizures, gout or any mental illness. The most common medications to trigger the condition are antibiotics, followed by analgesics (pain relievers), cough and cold medications, NSAIDs, anticonvulsants, and anti-gout drugs.
Drugs that may trigger a reaction includes
1) Antibiotics like penicillin
2) Medications to relieve and treat mental illness, seizures (anticonvulsants and antipsychotics)
3) Painkillers like ibuprofen, acetaminophen and naproxen sodium
4) Medications for gout therapy.
Infectious disease as triggers
1) Herpes virus infection from both types. herpes zoster and herpes simplex.
2) Pneumonia
3) HIV – an active HIV infection increases risk factor by 100 times for incidence of Stevenson’s Johnson syndrome.
4) Hepatitis
SIGNS AND SYMPTOMS
Often, Stevens-Johnson syndrome begins with flu-like symptoms, followed by a painful red or purplish rash that spreads and blisters, eventually causing the top layer of the skin to die and shed. To be classified as Stevens-Johnson syndrome, the condition must involve less than 10% of the body surface area.
The condition is characterized by painful, blistery lesions on the skin and the mucous membranes (the thin, moist tissues that line body cavities) of the mouth, throat, genital region, and eyelids. It can also cause serious eye problems, such as severe conjunctivitis; iritis, an inflammation inside the eye; corneal blisters and erosions; and corneal holes. Few of the symptoms we enlist
Fever, UNEXPLAINED MALAISE AND LETHARGY, Flu or flu type symptoms, Unexplained widespread skin patch, Blister on lips, mouth, nose, eyes and genitals, shedding of skin days after blister forms, Cough, Burning eyes or watery eyes, Sore throat, Acantholysis, Diarrhea, Macule, Weight loss
TREATMENT
The treatment is basically based upon identification of the cause if possible and immediately stopping the medication causing the reaction. Doctors will also try to relieve your symptoms, prevent infections, and support your healing. Replace the fluids and electrolytes if needed or management of other complications if arise so a proper uneventful healing takes place.
Antibiotics, corticosteroids, intravenous immunoglobulins can be administered if required. Although regrowth of the skin following Stevens-Johnson syndrome (SJS) is rapid (2-3 weeks), recovery can take weeks to months, depending on the severity of symptoms. Feelings of overwhelming tiredness may persist for months. Depression may also develop. For some, chronic complications may develop within weeks to months of an acute episode and can severely affect quality of life.
It is particularly important to spot the disease and manage it accordingly. prevention is cure and ignorance is not bliss in the case of this disease. Patient who are susceptible to the disease or having history of previous occurrences should be careful. They can get genetic screening done for HLA-B 1502 gene. Those who have history for reaction with certain drugs should avoid them to prevent the onset. The complications of Stevenson Johnson syndrome are quite severe resulting in sepsis, abscess, cellulitis, infection and inflammation in eyes, acute respiratory failure or permanent skin damage.
Having discussed the etiology, progress and related aspects of this rare yet dreadful disease, it is essential to reiterate the importance of avoiding the practice of self-prescription to ensure the health of our family members. It is always a good idea to seek help from trained healthcare professionals in cases of illnesses and any doubts. While maintaining a healthy lifestyle goes a long way in boosting our resistance to diseases, we should keep an eye open for the worst scenario by keeping ourselves informed about diseases like SJS.
