Stevens- Johnson's syndrome

Steven-Johnson's syndrome is a more serious form of erythema exsudativum multiforme which also affects the mucocutaneus transition areas (conjunctiva, lips and oral cavity, genitalia, rectum) with systemic manifestations. It is an immunopathological reaction in the skin that causes edema, epidermal necrosis and inflammatory vascular infiltration. Macroscopically it is manifested by an iris-shaped maculo-papullar rash (concentric circles) and blisters.

Etiology
The etiology is not always clear:
 * in children, the disease often follows the primary infection of herpes viruses, streptococcal infections or mycoplasma infections
 * in adults after administration of drugs, especially aminopenicillins or sulfonamides.

When a larger amount of body surface area is affected (> 30%), the condition is called Lyell's syndrome (toxic epidermal necrolysis).

Diagnostic criteria
These criteria are used to classify the Stevens-Johnson's syndrome :
 * mucosal inflications in two or more locations (conjunctiva, oral cavity and lips, genitals, anus),
 * small blisters affecting less than 10% of body surface area,
 * fever is present in 10-30% of all cases.

Therapy
In case of a concomitant course of primary infection with herpes viruses acyclovir, corticosteroids in high doses, or immunoglobulins are administered. The internal environment (dehydration, minerals) is monitored and affected parts of the skin are treated as burns, infectious complications are prevented.

Related articles

 * Erythema multiforme
 * Lyell's syndrome