Stevens Johnson Syndrome: Causes, Symptoms, Diagnosis, Complications, Treatment and Prevention

It arises as a hypersensitivity reaction, which generally affects the skin and mucous membranes.

Stevens Johnson syndrome represents a serious and potentially fatal mucocutaneous condition, characterized by an epidermal detachment, the presence of acute blisters on the skin , lesions or erosions on the mucous membranes.

In the Stevens Johnson syndrome, a more extensive involvement in the skin and mucosa is observed than in the case of erythema multiforme.

Initially, it was thought that erythema multiforme, Stevens Johnson syndrome and toxic epidermal necrolysis belonged to the same group of skin disorders.

But there is currently clinical evidence that erythema multiforme differs from the other two diseases.

The simplest classification of the disease is as follows:

  • Stevens-Johnson syndrome: It is a minimal form of toxic epidermal necrolysis, in which a detachment of less than 10% of the body surface is observed.
  • Superposition of Stevens-Johnson syndrome or toxic epidermal necrolysis:A detachment is observed that varies between 10 and 30% of the body surface area.
  • Toxic epidermal necrolysis: A detachment of more than 30% of the body surface area is observed.

Causes

In Stevens Johnson syndrome, the underlying cause is unknown in some cases.

However, there is in some people with Stevens Johnson syndrome a genetic predisposition to the disease, which increases the risk of developing it in response to precipitating factors.

The genetic mutation associated with Stevens Johnson syndrome occurs in the HLA-B gene.

This gene is part of the gene family called the human leukocyte antigen complex.

This gene complex reinforces the immune system, distinguishes proteins made by external invaders (such as viruses and bacteria).

Some viral infections that may precede this condition include herpes simplex or zoster, pneumonia , AIDS and hepatitis.

Likewise, it is usually caused by an allergic reaction to a medication.

The most common medications to trigger the condition are antibiotics, followed by analgesics, cough and cold medicines, non-steroidal anti-inflammatory drugs, anticonvulsants and medications for the treatment of gout. For example:

  • Medications for the treatment of gout and for a painful form of arthritis , such as: allopurinol (Aloprim, Zyloprim).
  • Analgesics such as acetaminophen, ibuprofen and naproxen sodium.
  • Antibiotics such as sulfonamides, are a type of drugs that fight infections (Bactrim and Septra).

symptom

The typical initial symptoms of Stevens Johnson syndrome are the following:

  • The presence of cough, producing a thick and purulent sputum.
  • Fevers of 39 to 40 ° C.
  • Sore throat.
  • Severe headaches
  • General discomfort.
  • Fatigue.
  • Pain in the bones and joints.

Then there is a fiery eruption in the patients that begins on the face and upper torso.

These skin lesions are characterized by the following:

  • The rash may start as spots that turn into blisters, confluent erythema, papules, urticarial plaques or vesicles.
  • In contrast to the typical lesions that appear with erythema multiforme, the lesions of the syndrome have only 2 areas of color.
  • The nucleus that presents the lesion can be vesicular, purple or necrotic and around the affected area there is inflammation and redness.
  • The lesions can become blisters and then rupture, leaving skin susceptible to secondary infections.
  • The lesions are edematous and usually do not cause itching.
  • Although the lesions usually appear anywhere on the body, the palms of the hands, the soles of the feet, the backs of the hands and the extensor surfaces are the most commonly affected.

The visible signs of mucosal involvement may include the following:

  • Redness of the skin.
  • Detachment of all those dead skin cells.
  • Fever.
  • Open injuries
  • Necrotic tissue

They are also observed with the help of the slit lamp, eye signs such as:

  • In the eyelids: Abnormal growth of the eyelashes such as: Trichiasis and distiquiasis, Meibomian gland dysfunction, inflammation of the eyelids.
  • In the conjunctiva: papillae, follicles, keratinizations, subepithelial fibrosis, conjunctival contraction, foreshortening of fornices, adhesion of the eyelid.
  • In the cornea: Inflammation of the cornea, epithelial defects, ulcers, neovascularization, keratitis, limbitis, conjunctivalization, perforations.

Diagnosis

For the differential diagnosis, the following must be done:

  1. A physical exam
  2. Skin biopsy
  3. Crops.
  4. Images.
  5. Blood test.
  6. Eye exam.

To rule out the presence of other diseases such as pneumonia, infections, erythema multiforme and toxic epidermal necrolysis

Complications

Some of the complications that occur as a result of suffering from Stevens-Johnson syndrome are:

  • Secondary infections in the skin: The appearance of infections in lesions, such as cellulite can cause complications that place life at risk.
  • Blood infections: Septic syndrome occurs when bacteria that are infecting an organ enter the bloodstream and spread throughout the body, causing shock and organ failure.
  • Eye problems: The rashes that Stevens Johnson syndrome induces can produce ocular inflammation. In mild cases, it can cause irritation and dry eyes, in severe cases, can cause extensive tissue damage and scarring that produces vision impairment or blindness.
  • Lung problems: This condition can cause acute respiratory failure.
  • Permanent damage to the skin: When the skin regenerates after suffering from Stevens Johnson syndrome, it may produce small bumps and abnormal coloring of the skin, it is possible that scars are formed.

Treatment

The treatment of patients with Stevens Johnson syndrome at the beginning does not differ from the therapy that is applied to patients suffering from extensive burns.

The respiratory tract, the hemodynamic stability, the state of fluids, the care of wounds and burns and the control of pain should be treated with special attention.

Generally, therapy for Stevens Johnson syndrome is carried out as follows:

  • Withdrawing any medication that is suspected of causing the disease is of paramount importance.
  • The treatment of oral injuries is done with mouthwash. The use of topical anesthetics are ideal to reduce pain and the administration of fluids to the patient is very important.
  • The areas of lacerated skin should be covered with compresses Burow solution (an aqueous solution with aluminum triacetate) or saline.
  • The usual prophylaxis in the treatment of tetanus should be addressed.

Among the recommended physical treatments, extensive debridement of the affected epidermis should be practiced, which will be covered immediately with biological bandages.

Treatment or ocular therapy of acute manifestations usually begins with a lubrication of the ocular surface.

As inflammation and scarring change occur, most ophthalmologists prescribe topical steroids, antibiotics, and mechanical lysis of eyelid adhesion.

In the case of presenting chronic superficial keratopathy, continuous lubrication may be sufficient.

When cases of ocular involvement are serious, the treatment then includes the following:

  • Elimination of keratinized plates from the margins of the eyelid.
  • Grafts of the mucous membrane, amniotic membrane grafts.
  • Stem cell transplantation of the eclero-corneal limbus of the healthy eye, amniotic membrane graft.
  • Surface keratectomy in order to eliminate the conjunctivalized or keratinized ocular surface.

Stevens Johnson syndrome can be difficult to treat.

And it is recommended that patients enter an intensive care or burn unit as soon as the diagnosis is suspected.

When severe symptoms occur, treatment may include:

  • Use of antibiotics to control any skin infection.
  • Prescription of corticosteroids for the control of inflammation.
  • Treatment for the eyes may include the use of artificial tears, antibiotics or corticosteroids.

Risk factor’s

You are more likely to have Stevens Johnson syndrome if the patient has the following risk factors:

  • AIDS or some other problem of the immune system.
  • Have suffered from Stevens Johnson syndrome previously.
  • Possess the genetic tendency.
  • Undergo treatments with radiations.
  • Possess Asian descent.

Prevention

There is no way to know how the body will react to the ingestion of certain medications, but if you have already had the disease, you must be very careful, you must know the risk factors.

  • At the time of prescribing any medication the patient must inform in the clinical history that he suffered from Stevens Johnson syndrome.
  • The patient should wear a medical alert bracelet and know the name of the medication that gave rise to the syndrome.

Forecast

The recovery of the skin lasts 2 to 3 weeks, the general recovery can take from weeks to months, depending on the severity with which the symptoms occurred.

The symptoms of fatigue, depression, can persist for months, complications can develop weeks and months affecting the quality of life.

Recurrence of Stevens Johnson syndrome varies between 3 to 7% for adults and 7 to 18% for children and the risk of recurrence is greater when Stevens Johnson syndrome is associated with an infection or an unknown cause.

Recurrence in Stevens Johnson syndrome associated with the reaction to a medication can only occur if the patient is given the same trigger drug or one related to the component.

In some patients, Stevens Johnson syndrome can be fatal.