Steven-Johnson Syndrome (SJS) is a severe allergic cutaneous disorder. This skin disorder is often brought about by the intake of common drugs like sulfa drugs, anti-convulsants, and antibiotics. The syndrome is characterized by generalized macular lesions that rapidly spread and consolidate leading to epidermal necrosis, blistering and epithelial sloughing.
Constitutional signs of fever, fatigue, coughing, and sore throat may be observed several days before cutaneous signs develop among patients with SJS. The following signs and symptoms are seen among patients suffering from SJS:
Patients are systematically examined physically for the eminent cutaneous lesion and associated signs of SJS. A detailed clinical history on the onset of the symptomatology will easily grasp the diagnosis for SJS. A skin biopsy is frequently done to definitively diagnose SJS in suspected patients.
The most important approach in the treatment of SJS is the sudden discontinuation of the inciting factor implicated in the initiation of the immune response. In cases where the inciting drug is not identified, physicians will usually ask their patients to discontinue all non-essential drugs to eliminate the possible causative agent. SJS is a medical emergency, thus all patients are managed promptly in the hospital to stabilize fluid imbalance and correct nutritional deficiencies. Adequate wound care and eye care may be in place where another specialist may be brought in for comanagement .
Antiinflammatory agents to control inflammation, itching, and pain may be used to allay these symptoms. Corticosteroid may also be given as an immune modulator agent for SJS . Patients are given antibiotic therapy to control concurrent infections associated with the cutaneous disease.
Cutaneous lesion of mild SJS typically heals within 1 to 2 weeks from onset without any serious sequelae. However, this may be complicated by secondary skin infections that increases the morbidity rates among afflicted patients. The mortality rate in SJS varies directly with the body surface area (BSA) of the cutaneous sloughing. Mortality rates may climb beyond 50% for BSA percentage of 25% to 35% of skin affected . Patients who survive SJS may develop a number of sequelae in some cases, these include photophobia, watery eyes, inverted eyelashes, and burning sensation of the eyes.
In more than half the cases of SJS, drugs are commonly considered as the precipitating agent. The following are the most common drugs in the development of the severe cutaneous reaction in SJS:
There are other factors that may incite the hypersensitivity reaction in SJS, these are infections (most commonly by Mycoplasma pneumonia), Graft rejection disease, and vaccination reactions. Studies have demonstrated that patients with systemic lupus erythematosus (SLE) have an increased risk in developing SJS . In some instances, the true etiology of SJS remains unknown or idiopathic in nature.
In the United States, medic aid data has determined that the incidence of SJS has reached to 6.8 cases per million population per annum . The majority of cases of SJS have a predilection to the winter and early spring seasons. Sulfonamides and piroxicam are the most common inciting factors implicated in the US, while allopurinol is more common in Southeast Asian nations like Malaysia and Hong Kong. SJS occurs in all races but is more commonly seen in the white races compared to their dark skinned counterparts. Large cohort studies have determined that the mean age of onset in SJS is 25 years old and this may occur in children as young as 3 years old.
The pathophysiology in SJS is described as an idiosyncratic and delayed hypersensitivity reaction to an inciting factor. There are certain sectors of the population who are more prone to the disease inclusive are those in immunocompromised states (i.e. AIDS) and those under radiotherapy for cancer . The antigen presentation and the production of acute phase reactants (like tumor necrosis factor) by the tissue dendrocytes will advertently result in the recruitment and the proliferation of the T-lymphocytes which enhances cytotoxicity .
There is a subsequent death in the keratocytes that leads to the separation of the dermis and the epidermis that clinically present as sloughing. The perpetuation of the inflammation by the chemokines leads to extensive epidermal necrolysis of the surface epithelium . Inciting factors may be discontinued to stop and reduce the inflammatory process or may be titrated in smaller divided doses if drug is absolutely necessary and essential.
Patients who have Asian lineage are perpetually advised to undergo genetic testing for SJS before starting on carbamazepine indicated for the control epilepsy. Patients who have had SJS in the past must avoid by all means to take in the identified inciting agent to avoid its recurrence. In the same way, family members are also discouraged to ingest the inciting drug because of the genetic risk factors involved.
Steven-Johnson syndrome (SJS) is a rare and life-threatening disorder of the skin and the mucous membrane . This syndrome is usually a reaction to an infection or an intake of a medication that starts out as a reddish rash that disseminates in the skin surface and forms blisters. SJS is considered as a medical emergency that requires prompt treatment focusing on the elimination of the causative agent, the control of the symptoms, and preventing further complications.
Steven-Johnson syndrome is a rare and life-threatening disorder of the skin and the mucous membrane described to exaggerated hypersensitivity response to an inciting factor.
More than half the cases of SJS are initiated by the intake of drugs like sulfa drugs, antibiotics, anticonvulsants, and analgesics. Less common causes includes infections, graft rejection reactions and vaccinations.
Patients will present with severe skin and mucous membrane lesions which are erythematous, itchy, and blistering.
A detailed clinical history and a meticulous physical examination of the skin may secure the diagnosis of SJS. Skin biopsy is the definitive test used for this kind of disease.
Treatment and follow-up
Treatment focuses on the prompt removal of the inciting factor, the stabilization of the patient through fluid resuscitation, and the control of the signs and symptoms.