Stephen Johnson's Syndrome description

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For those of you not familiar with this :

 

Background: Stevens-Johnson syndrome (SJS) is an immune-complex–mediated hypersensitivity complex that is a severe expression of erythema multiforme. It is now known also as erythema multiforme major. SJS typically involves the skin and the mucous membranes. While minor presentations may occur, significant involvement of oral, nasal, eye, vaginal, urethral, GI, and lower respiratory tract mucous membranes may develop in the course of the illness. GI and respiratory involvement may progress to necrosis. SJS is a serious systemic disorder with the potential for severe morbidity and even death.

Pathophysiology: SJS is an immune-complex–mediated hypersensitivity disorder that may be caused by many drugs, viral infections, and malignancies. Cocaine recently has been added to the list of drugs capable of producing the syndrome. In up to half of cases, no specific etiology has been identified.

History:

 

Typically, the disease process begins with a nonspecific upper respiratory tract infection.

 

 

This usually is part of a 1- to 14-day prodrome during which fever, sore throat, chills, headache, and malaise may be present.

 

 

Vomiting and diarrhea occasionally are noted as part of the prodrome.

 

Mucocutaneous lesions develop abruptly. Clusters of outbreaks last from 2-4 weeks. The lesions typically are nonpruritic.

 

A history of fever or localized worsening should suggest a superimposed infection; however, fever has been reported to occur in up to 85% of cases.

 

Involvement of oral and/or mucous membranes may be severe enough that patients may not be able to eat or drink.

 

Patients with genitourinary involvement may complain of dysuria or an inability to void.

 

A history of a previous outbreak of SJS or of erythema multiforme may be elicited. Recurrences may occur if the responsible agent is not eliminated or if the patient is reexposed.

 

Typical symptoms are as follows:

 

 

Cough productive of a thick purulent sputum

 

 

Headache

 

 

Malaise

 

 

Arthralgia

Physical:

 

The rash can begin as macules that develop into papules, vesicles, bullae, urticarial plaques, or confluent erythema.

 

 

The center of these lesions may be vesicular, purpuric, or necrotic.

 

 

The typical lesion has the appearance of a target. The target is considered pathognomonic.

 

 

Lesions may become bullous and later rupture, leaving denuded skin. The skin becomes susceptible to secondary infection.

 

 

Urticarial lesions typically are not pruritic.

 

 

Infection may be responsible for the scarring associated with morbidity.

 

 

Although lesions may occur anywhere, the palms, soles, dorsum of hands, and extensor surfaces are most commonly affected.

 

 

The rash may be confined to any one area of the body, most often the trunk.

 

 

Mucosal involvement may include erythema, edema, sloughing, blistering, ulceration, and necrosis.

 

The following signs may be noted on examination:

 

 

Fever

 

 

Orthostasis

 

 

Tachycardia

 

 

Hypotension

 

 

Altered level of consciousness

 

 

Epistaxis

 

 

Conjunctivitis

 

 

Corneal ulcerations

 

 

Erosive vulvovaginitis or balanitis

 

 

Seizures

Coma

Causes:

 

Drugs and malignancies most often are implicated as the etiology in adults and the elderly.

 

Pediatric cases are related more often to infections than to malignancy or a reaction to a drug.

 

A medication such as sulfa, phenytoin, or penicillin had previously been prescribed to more than two thirds of all patients with SJS.

 

More than half of the patients with SJS report a recent upper respiratory tract infection.

 

The 4 etiologic categories are (1) infectious, (2) drug-induced, (3) malignancy-related, and (4) idiopathic.

 

 

Infectious diseases that have been reported include herpes simplex virus (HSV), influenza, mumps, cat-scratch fever, mycoplasmal infection, lymphogranuloma venereum (LGV), histoplasmosis, and cholera.

 

 

In children, Epstein-Barr virus and enteroviruses have been identified.

 

 

Drug etiologies include penicillins, sulfas, phenytoin (and related anticonvulsants), carbamazepine, and barbiturates. In late 2002, the US Food and Drug Administration (FDA) and the manufacturer Pharmacia noted that SJS had been reported in patients taking the cyclooxygenase-2 (COX-2) inhibitor valdecoxib.

 

 

Various carcinomas and lymphomas have been associated.

 

 

SJS is idiopathic in 25-50% of cases.