Stevens-Johnson Syndrome Disease

DESCRIPTION

Until recently Stevens-Johnson syndrome (SJS) was considered to be the same as erythema multiforme major, a severe form of erythema multiforme in which more than one mucosal surface was involved. Now it is thought that erythema multiforme spectrum is a single entity. The milder form, also known as erythema multiforme-Hebra, either has no mucous membrane involvement, or may involve one mucous membrane. The more severe form is erythema multiforme major, involving more than one mucus membrane. In both these variants, it is a self limited hypersensitivity reaction, usually to a preceding viral infection, and has an excellent prognosis. SJS is a generalized hypersensitivity reaction, usually to a drug, in which the skin and mucus membrane lesions are early manifestation. It may progress to its more severe form, toxic epidermal necrolysis which has a high morbidity and up to 40% mortality.

System(s) affected: Skin/exocrine, Nervous, Cardiovascular, Renal/Urologic, Hemic/Lymphatic/Immunologic
Genetics: Possibly associated with HLA-B15
Incidence/Prevalence in USA: Difficult to estimate because of confusion with erythema multiforme major, perhaps 0.1/100,000, or less.
Predominant age: More common in children and young adults
Predominant sex: Males > Females (2:1)

SIGNS AND SYMPTOMS

There is usually a preceding illness for which medication was given
Sudden onset with rapid progressive pleomorphic rash which includes petechiae, vesicles, bullae
The condition is classified as SJS if epidermal detachment affects less than 10% of the skin, as toxic epidermal necrolysis (TEN) if epidermal detachment exceeds 30%, or if it exceeds 10% in the absence of discrete skin lesions. Cases with discrete skin lesions and between 10% and 30% epidermal detachment are in the overlap between SJS and TEN.
Vesicles and ulcers on the mucous membranes, especially of the mouth and throat
Burning sensation of the skin and sometimes of the mucous membranes
Usually no pruritus
Fever 39-40°C (102-104°F)
Headache
Malaise
Arthralgias
Epistaxis
Crusted nares
Conjunctivitis
Corneal ulcerations
Erosive vulvovaginitis or balanitis
Cough productive of thick purulent sputum
Tachypnea/respiratory distress
Albuminuria/hematuria
Arrhythmias
Pericarditis
Congestive heart failure
Mental status changes
Electrolyte disturbance
Seizures
Coma
Sepsis

CAUSES

Often unknown
Medications - especially sulfonamides, penicillins, anticonvulsants, salicylates, nonsteroidal anti-inflammatory drugs, methazolamide, carvedilol (3,4)
Vaccines - diphtheria/typhoid, bacillus Calmette Guerin (BCG), oral polio vaccine (OPV)
Mycoplasma pneumonia virus infection

RISK FACTORS

Patients with HIV infection appear to be predisposed to developing SJS in response to their medications
Previous history of SJS
Male sex

LABORATORY

Culture or serological tests for suspected sources of infection

Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS

Compared with the mainly inflammatory changes in erythema multiforme, necrotic changes predominate in SJS and TEN. There is a cell poor infiltrate in which macrophages and dendrocytes predominate with a strong immunoreactivity for TNF-alpha.

SPECIAL TESTS

None

IMAGING

N/A

DIAGNOSTIC PROCEDURES

Skin biopsy

APPROPRIATE HEALTH CARE

Since this disease can progress quickly, all patients should be admitted. If the sloughed skin exceeds 10% of the body surface, consideration should be given to transferring the patient to a burns unit. Bronchiolitis, adult respiratory distress syndrome or multi-organ damage may require care in an intensive care unit.

GENERAL MEASURES

Withdrawal of any suspected medication, and treatment of any underlying disease
Meticulous care of damaged skin
Reverse isolation when epidermal loss is extensive
Maintenance of fluid, electrolyte and protein balance
Adequate calorie intake, parenteral nutrition if necessary
Oral hygiene with mouthwashes of warm saline, or solution of diphenhydramine, lidocaine, and kaolin suspension
Ophthalmological consultation and monitoring for corneal damage

SURGICAL MEASURES

Sterile débridement of areas of extensive epidermal loss. Application of biosynthetic dressings such as Biobrane to denuded areas (13 ) Long term damage to the vulva or vagina or to the cornea or may need surgical repair.

ACTIVITY

Bed rest until clinically stabilized

DIET

As tolerated. Increased fluid intake is recommended. Intravenous nutritional support may be needed.

PATIENT EDUCATION

The patient should be kept informed of the progress of the disease, and the treatment options available
Recurrences are possible. Etiologic agents should be identified if possible, and avoided indefinitely.

PREVENTION/AVOIDANCE

It is rarely possible to anticipate a first attack
Avoid reexposure to the presumed cause

POSSIBLE COMPLICATIONS

Secondary infections
Sepsis
Pneumonia
Adult respiratory distress syndrome
Bronchiolitis obliterans in children
Dehydration/electrolyte disturbance
Acute tubular necrosis
Corneal ulceration or iritis
Arrhythmias
Death in about 15% of untreated cases of SJS, and up to 40% of TEN

EXPECTED COURSE AND PROGNOSIS

Disease may have a rapid onset, or may evolve slowly over 1-2 weeks, with resolution over 4 to 6 weeks
There may be scarring of the skin or mucous membranes, especially of the vulva
There may be blindness or corneal opacities in 7-20% of patients
Risk of recurrence may be as high as 37%
Death occurs in 5-15% of patients with SJS, and up to 40% of patients with TEN

ASSOCIATED CONDITIONS

Stevens-Johnson syndrome and toxic epidermal necrolysis are associated conditions
Mycoplasma pneumonia has been described as a viral precursor

AGE-RELATED FACTORS

Pediatric: Rare under 3 years. More common in children and young adults
Geriatric: TEN has a greater mortality in older patients
Others: N/A

PREGNANCY

Reported as a possible predisposing condition

OTHER NOTES

N/A

ABBREVIATIONS

N/A

Clinical Investigations

ROLE OF HOMOEOPATHY

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