Introduction:
Stevens-Johnson syndrome is a very severe erythema multiforme. Erythema multiforme is a skin condition with a reddish or purplish lesion. Stevens-Johnson syndrome appears in the form of blisters. These blisters are known as bullous forms of SJS, and they have widespread involvement in the skin, oral cavity, eyes, and genitals. It begins with 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.
What Is Stevens-Johnson Syndrome?
Steven-Johnson syndrome is a rare serious skin disease caused due to a reaction of medicines or infections. An individual may have flu-like symptoms, rashes, blisters, and skin shedding. The condition is life-threatening, so it requires immediate medical conditions.
Whom Does Stevens-Johnson Syndrome Affect?
Stevens-Johnson syndrome is a sporadic 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 (acquired immunodeficiency syndrome). 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 destroys the whole immune-mediated functions, giving way to a lot of opportunistic infections. These patients have to stay extra careful. Infections like pneumonia can also be a contributing factor to Stevens-Johnson syndrome.
What Are the Causes of Stevens-Johnson Syndrome?
Stevens-Johnson syndrome is 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 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 Risk Factors for Stevens-Johnson Syndrome?
Stevens-Johnson syndrome has the following risk factors:
-
Viral Infections: Viral infections such as herpes, viral pneumonia, hepatitis, and HIV (human immunodeficiency virus).
-
Weakened Immune System: This may be the result of HIV or AIDS, lupus, or autoimmune conditions.
-
A 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 Symptoms of Stevens-Johnson Syndrome?
Stevens-Johnson syndrome shows changes in different body regions, especially in the mucous membranes and the skin. The mucous membranes that have minimum amounts of moisture are generally affected. The commonly involved mucous membranes are the eyes, oral cavity, and genitals. Flu-like symptoms might be seen. They include fever, cough, and headache. In severe cases, it might also involve hair and nails.
-
General Symptoms: The symptoms include fever, skin pain, body ache, red rash on the skin, cough, blisters and sores on the skin, and painful urination.
-
Eye Lesions: Stevens-Johnson syndrome causes photophobia. 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:
-
Urethritis: It is the inflammation of the urinary tract. It may lead to urinary retention.
-
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.
-
Vaginal Ulcers: They are very painful lesions and may lead to stenosis if not managed properly.
-
What Are the Oral Manifestations of Stevens-Johnson Syndrome?
Oral mucous membrane lesions may be extremely severe and so painful that even chewing becomes impossible. The mucosal vesicles or blisters rupture 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.
How Is Stevens-Johnson Syndrome Diagnosed?
A dermatologist diagnoses Stevens-Johnson syndrome. Only these skin specialists can identify the different forms of skin problems. Diagnosis is made 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 Treatment Option for Stevens-Johnson Syndrome?
Identification of the cause should be made. 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. Using liquid antiseptics, such as 0.05 percent Chlorhexidine, during bathing is preferable. Systemic corticosteroids are controversial, and some believe they may predispose to complications.
What Are the Complications of Stevens-Johnson Syndrome?
The complications of stevens-johnson syndrome are as follows:
-
Sepsis (a life-threatening condition causing systemic inflammation and organ dysfunction).
-
Scarring on rash and blister areas in acute phases.
-
Eye complications cause conjunctivitis, corneal ulceration, and vision loss.
-
Respiratory complications, such as pneumonia and acute respiratory distress syndrome (serious lung condition).
-
Difficulty in swallowing due to scarring of the esophagus.
-
Secondary infections.
-
Systemic complications, such as liver dysfunction and renal failure.
-
Psychological impact, such as anxiety and depression.
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 an individual had Stevens-Johnson syndrome earlier, they might be at risk of getting affected again.
Conclusion:
Steven-Johnson syndrome is a skin disease affecting the mucous membrane. Treatment involves discontinuing causative medications, fluid replacement, and supportive care to manage symptoms. It can cause several complications, such as scarring and eye and respiratory issues. The healthcare provider may recognize the condition early, with prompt intervention and comprehensive management. In addition, patient education and supportive care are crucial for managing physical and psychological effects.
