Stevens Johnson Syndrome

This syndrome has been named after the pediatricians AM Stevens and SC Johnson who were the first to diagnose a reaction of eye and mouth due to a drug in the year 1922. It is a rare disorder or condition but does serious damage to the mucus membranes besides the skin. Abbreviated as SJS, it is a medical emergency known as toxic epidermal necrolysis which requires immediate attention and medical care as it is known to be one of the most adverse drug reactions recognized in USA. The affected groups include Elderly and the children.

This condition causes swelling of face and lips with red and purple rash that blisters on the face accompanied by crusty lip sores. In case the eyes catch it then it can cause vision issues and corneal ulcers.

Genetic Issues – If a close family member has suffered from SJS, there is high possibility of SJS risk. There is a strong connection particularly with the Asian population between HLA-A 33:03 and HLA-C 03:03 alleles as well as allopurinol induced SJS or TEN.

Causes because of medication reactions
Usually the reaction to a certain drug appears within a week of starting the course but with some medications like anti-convulsants, the reaction might take as long as a month to surface. The antibiotics that are particularly on the list to cause SJS are Penicillins like bacampicillin and amoxicillin, cephalosporins like cephalexin and cefaclor, macrolides like azithromyvin, erythromycin and clarithromycin. Some anticonvulsantsare used for treating peilepsy, allopurinol used for gout treatment, Nevirapine for treating HIV infections, Acetaminphen, an all age group medication.

Immunity Issues – If you have an immune system that is weak like HIV infection, due to organ transplant or chemotherapy and more could increase the risk of SJS

Infections that Predispose to SJS
The viral infections that are known to precede SJS include influenza, Hepatitis A, mums, HIV and more while the bacterial include conditions like typhoid, brucellosis, mycoplasma pneumonia to name a few. Malaria and trichomoniasis are the protozoal infections.

Previous infections – There is a high risk of SJS recurring if the same medication of the same group has been used earlier and has been responsible for causing SJS infection.

Symptoms
It primarily looks like an upper respiratory tract infection, cold or cough, headache, body pain and sore throat
It is followed by blisters on the skin, mouth, nose, ears and genitals as well. The rashes are red or purple in color and with the progress of the conditons may tend to merge as well as rupture thus exposing sores that are painful. Further, the top skin layer forms a crust and sheds.

The face and lips swell and mouth catches ulcers. The lips form a crusty sore and due to the ulcer buildup in the mouth, its becomes difficult to swallow. If there are ulcers in the digestive tract, they may cause diarrhea and even dehydration.

Diagnosis
There are two ways by which the Stevens Johnson syndrome can be diagnosed.
1. Clinical Examination – The target rash characteristic plays an important role in diagnosis of SJS but to study the predisposing factor a complete clinical history is checked.
2. Skin biopsy – Sometimes even a skin biopsy could be needed to confirm the diagnosis.

Treatment -

The patient needs immediate hospitalization because it is considered to be a medical emergency. The first step is to stop the medications responsible for SJS. For treating the infection that is already developing, local antiseptics and antibiotics are used.

To stop the condition from getting worse and spreading, intravenous immunoglobulins are given.
The eye treatment involves artificial tears and lubricants so that the surface drying stops. Even antibiotics, antiseptics and topical fluids are used in order to prevent inflammation as well as infection.
For women suffering from the condition intravaginal steriod oinments are used for preventing scar tissue formation
Painkillers/ Analgesics for relief
Psychological consultancy for emotional stability and anxiety if the need be.

Prevention
If some close relative has developed SJS its important to know the drug that caused it and try and avoid that drug so as to prevent it
These days medicalert bracelets are available so that the health professional who treats come to know about any emergency situation.

Genetic testing is rarely carried out and might be suggested only in cases of South East Asian and Indian descent.
It is known that of the one third cases diagnosed with Stevens Johnson syndrome, the recurrence does occur and hence it is important to stay informed about the medications that might just not be suitable for intake for this condition.

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*Source from the internet