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Stevens-Johnson Syndrome - Causes, Symptoms, and Treatment

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Stevens-Johnson Syndrome - Causes, Symptoms, and Treatment

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Stevens-Johnson syndrome is a rare skin disease. Read this article to know more about the causes, symptoms, and treatment.

Written by

Dr. Lochana .k

Medically reviewed by

Dr. Shama Naaz

Published At November 24, 2020
Reviewed AtOctober 18, 2022

Stevens-Johnson syndrome is a very severe erythema multiforme. Erythema multiforme is a skin condition that presents as a reddish or purplish lesion. Stevens-Johnson syndrome appears in the form of blisters. These blisters are known as bullous form of SJS, and it has widespread involvement of the skin, oral cavity, eyes, and genitals. It begins with the many abrupt symptoms such as fever, malaise, photophobia, and eruptions of the oral mucosa, genitalia, and skin. The skin lesions are commonly accompanied by a discharge of the blood from the lesions. They are often vesicular or bullous.

Whom Does It Affect?

Stevens-Johnson syndrome is a very rare disease. It affects one or two individuals out of a million people each year. Children and adults who are less than 30 years of age are commonly affected. Women are affected more than men. Certain medical conditions make an individual more prone to Stevens-Johnson syndrome. They are systemic lupus, chronic inflammatory diseases, and AIDS. The high risk for Stevens-Johnson syndrome in these patients is not clear. It might be due to the poor immune functions seen in these patients. Generally, AIDS causes destruction of the whole immune-mediated functions, giving way for a lot of opportunistic infections. These patients have to stay extra-careful. Infections like pneumonia can also be a contributing factor for Stevens-Johnson syndrome.

What Are the Symptoms?

TheStevens-Johnson syndrome shows changes in different regions of the body, especially in the mucous membranes and the skin. The mucous membranes that are having minimum amounts of moisture are generally affected. The commonly involved mucous membranes are eyes, oral cavity, and genitals. The flu-like symptoms might be seen. It includes fever, cough, and headache. It might also involve hair and nails in severe cases.

  • Eye Lesions: Stevens-Johnson syndrome causesphotophobia. Patients with photophobia experience pain or discomfort when exposed to light. The other symptoms are conjunctivitis and corneal ulceration. Conjunctivitis and corneal ulcerations are eye infections. Corneal ulceration is a painful condition affecting the cornea. In addition to these, the other eye lesion is panophthalmitis. Panophthalmitis is a sudden infection affecting all parts of the eyeball. Some severe bacterial infections can lead to blindness.

  • Genital Lesions: Genital lesions involve the following conditions:

  1. Urethritis. It is the inflammation of the urinary tract. It may lead to urinary retention.

  2. Balanitis. It is the inflammation of the head of the penis along with the foreskin. It is often seen along with a foul-smelling discharge due to bacterial infection.

  3. Vaginal ulcers. They are very painful lesions and may lead to stenosis, if not managed properly.

What Are the Oral Manifestations?

Oral mucous membrane lesions may be extremely severe and so painful that even chewing becomes impossible. The mucosal vesicles or blisters ruptures and leave the surface covered with a thick white or yellow form of exudate. Erosions of the pharynx are also common. The lips may exhibit ulceration with bloody crusting and are painful.

What Are the Risk Factors?

Stevens-Johnson syndrome has the following risk factors:

  • Viral infections: Viral infections such as herpes, viral pneumonia, hepatitis and HIV.

  • Weakened immune system: This may be the result of HIV or AIDS, lupus, autoimmune conditions.

  • A past history of Stevens-Johnson syndrome.

  • A family history of Stevens-Johnson syndrome.

  • Certain genes are known to increase the risk of Stevens-Johnson syndrome.

  • Chronic diseases of joints.

  • Chronic diseases of the connective tissue.

What Are the Causes of Stevens-Johnson Syndrome?

Many causes of Stevens-Johnson syndrome are caused by medications causing an allergic reaction. The exact cause of this condition is not known. Researchers are still trying to identify the exact cause of this disease. When a medication is known to cause allergy in a person, the symptoms begin one or three weeks after the intake of medicines.

More drugs are listed to be the cause of Stevens-Johnson syndrome. Some of them are:

  • Medicines that are prescribed for the treatment of gout.

  • Pain relievers such as Acetaminophen and Ibuprofen.

  • Allopurinol drugs that are given for the painful form of arthritis.

  • Medicines that are used to treat seizures or any other mental illness.

  • Certain antibiotics.

What Are the Complications?

Other related complications are related to respiratory tract involvement, such as tracheobronchial ulceration and pneumonia. The patients usually recover unless they succumb to a secondary infection.

How Is Stevens-Johnson Syndrome Diagnosed?

Stevens-Johnson syndrome is diagnosed by a dermatologist. Only these skin specialists will be able to identify the different forms of skin problems. Diagnosis is done after receiving a detailed history from the patient regarding the past illnesses experienced by the patient and also the family history mentioning the health conditions of the siblings, parents, and blood relatives. A confirmatory test might require a biopsy sample. The biopsy result will provide a more detailed description of the condition.

What Is the Differential Diagnosis?

The varied nature of the disease may present difficulty in diagnosis, particularly when the occurrence of the skin lesions is minimal. In the presence of oral lesions, aphthous stomatitis, contact dermatitis or stomatitis, and acute necrotizing gingivitis must be considered, as well as pemphigus, dermatitis herpetiformis, bullous lichen planus, herpes zoster, chickenpox, and toxic epidermal necrolysis (Lyell’s disease).

What Is the Treatment Option?

Identification of the cause should be made if possible. If a drug is suspected, it must be withdrawn. Infections should be appropriately treated after cultures. Serologic tests must be performed often. For Stevens-Johnson syndrome, symptomatic treatment is required, including oral antihistamines, analgesics (only if it is not an offending drug), local skincare, and soothing mouthwashes. Topical steroids may be considered. Oral antacids may be helpful for discrete oral ulcers. The use of liquid antiseptics, such as 0.05% Chlorhexidine, during bathing, is preferable. Systemic corticosteroids are controversial, and some believe they may predispose to complications.

What Are the Prevention Measures for Stevens-Johnson Syndrome?

If Stevens-Johnson syndrome is caused due to any medications, avoid taking that particular medication. The patient’s family members also should avoid taking these medications in case of genetic susceptibility within the family. If you had Stevens-Johnson syndrome earlier, you might be at risk of getting affected again. So always look for symptoms and take more care of yourself.

Frequently Asked Questions


Can People With Stevens-Johnson Syndrome Survive?

Skin and mucous membranes are affected by the uncommon, dangerous illness known as Stevens-Johnson syndrome (SJS). If this ailment is not treated properly, it could be fatal. Depending on the severity of their ailment and how soon they receive treatment, people with SJS may have a wide range of prognoses. While some SJS patients may recover, others may experience long-term problems.


Will Stevens-Johnson Syndrome Spread From Person to Person?

Stevens-Johnson syndrome is not a condition that spreads easily. A rare and deadly illness results when the immune system destroys healthy cells. Several things can bring it on, such as specific drugs, infections, and underlying medical disorders. Unlike illnesses, the condition does not transmit from person to person.


What Area Does the Stevens-Johnson Syndrome Rash Begin?

It begins with flu-like symptoms, including fever and body aches, and develops into a rash over time. Usually, the face and trunk are where the rash first appears before moving to other body areas. Small, red patches that later become painful, blistering sores may first appear. In severe cases, the skin may peel off, exposing raw, exposed areas vulnerable to infection. The rash can affect the skin of the face, lips, and genitals.


Does COVID Cause Steven-Johnson Syndrome in Individuals?

Some cases reported that covid affected individuals are also affected with Stevens-Johnson syndrome. But no correct evidence and reports suggest COVID and the occurrence of Stevens-Johnson syndrome.


Will Stevens-Johnson Syndrome Appear Suddenly?

Yes, Stevens-Johnson syndrome (SJS) often starts suddenly, with symptoms appearing over a few days or weeks. The most common initial symptoms are flu-like, such as fever, cough, and body aches. Other early symptoms may include a red or purple rash, blisters on the skin and mucous membranes (such as the mouth, nose, and eyes), and painful sores on the skin and mucous membranes.


Will Stevens-Johnson Syndrome Appear After Vaccination?

This serious condition can result from an adverse reaction to a medication or vaccine. It is characterized by a rash, blisters, and skin detachment, and it can be life-threatening if not treated promptly. The risk of developing SJS after vaccination is very low, but it is possible. The risk of developing SJS after receiving a vaccine is estimated to be less than 1 in a million doses.


Is Stevens-Johnson Syndrome a Medical Emergency?

Stevens-Johnson syndrome is life-threatening and requires immediate medical attention. It is a type of severe skin reaction that can cause the skin to blister and peel off, exposing raw areas on the body. SJS can also affect the mucous membranes, including the eyes, mouth, and genital area. If left untreated, it can be dangerous.


What Are the Diagnostic Tests for Stevens-Johnson Syndrome?

A healthcare provider will perform a physical examination to diagnose SJS and ask about your medical history, including any medications you are taking. They may also order tests to help confirm the diagnosis, such as blood tests
, skin biopsy, and culture of blister fluid or mucous membranes.


What Is the Duration of Stevens-Johnson Syndrome to Progress?

The Stevens-Johnson syndrome progression begins within eight weeks following the first exposure to the causative factor. Only in rare cases do the symptoms appear within a few hours.


Are There Any Mild Forms of Stevens-Johnson Syndrome?

There is no mild form of SJS. However, mild cases may be classified as a less severe condition called toxic epidermal necrolysis (TEN). TEN is similar to SJS but less severe and affects a larger body area. SJS and TEN are considered medical emergencies and should be treated as soon as possible.


Will Stevens-Johnson Syndrome Symptoms Subside?

If SJS is diagnosed and treated early, the prognosis is generally good. Treatment may include hospitalization, medications to control the immune system's response, and supportive care such as wound care and fluids. SJS can leave long-term effects, such as scarring and changes in skin color, and in some cases, it can cause damage to the eyes and other organs. However, most people with SJS can recover fully with proper treatment and follow-up care.


Will Ibuprofen Medication Cause Stevens-Johnson Syndrome?

Ibuprofen medication is a nonsteroidal anti-inflammatory drug (NSAID) and can be associated with the development of SJS in some people. However, it is important to note that SJS is rare, and the risk of developing SJS from ibuprofen is very low. If rash and breathing difficulty occurs after consuming this medicine, immediately stop taking this and seek immediate medical attention.


Which Organs Are Affected Due to Stevens-Johnson Syndrome?

Stevens-Johnson can affect any organ in the body, but it most commonly affects the skin, eyes, mouth, and genitals. It can also affect the respiratory, digestive, and urinary tracts. In severe cases, SJS can be life-threatening, as it can lead to organ failure and other complications.


What Is the Survival Rate of Stevens-Johnson Syndrome-Affected Individuals?

The survival rate depends mostly on the condition's severity and the individual's overall health. The survival rate for SJS is generally high, but it can be associated with serious complications and a long recovery process. Some individuals may have long-term complications or permanent damage to the skin, eyes, and other organs. There is a need for immediate medical attention in such cases.
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Dr. Shama Naaz
Dr. Shama Naaz



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