Stevens-Johnson syndrome is a rare skin disease. Read this article to know more about the causes, symptoms, and treatment.
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.
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.
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:
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.
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.
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.
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.
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.
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.
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).
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.
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.
Last reviewed at:
25 Nov 2020 - 4 min read
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