HEALTHNET

Steven-Johnson Syndrome (SJS) - Part I

SJS is a serious systemic bodywide allergic reaction with a characteristic rash of iris lesions and target lesions involving the skin and mucous membranes...

(Posted on 12 July 2007)

Steven-Johnson Syndrome (SJS) was first described by American pediatricians, Dr. Albert Mason Stevens and Frank Chambliss Johnson in 1920. SJS is a serious systemic bodywide allergic reaction with a characteristic rash of iris lesions and target lesions involving the skin and mucous membranes of the oral, nasal, ocular, genitourinary, and anal areas etc. More than 50% of the reported cases were caused by a reaction to a drug, where the others derive from infections (e.g. HIV, fungal infections) and reduced immunity. However, the distinct underlying cause of SJS remains unknown. So far there are approximately over 100 drugs reported to be capable of eliciting SJS. The list of drugs which are known to commonly cause SJS are shown in table 1. The incidence of SJS is rare with approximately 1 to 6 reported cases per 1,000,000 population, and its onset is irrespective to age, race, sex, and seasons.

The disease process for SJS typically begins with a nonspecific upper respiratory tract infection, and manifestation of symptoms during onset such as fever, cough, malaise, chest pain etc. Vomiting and diarrhea are sometimes noted in the early symptoms. Continued fever, fatigue, and clusters of outbreaks of skin and mucous membrane lesions involving the conjunctiva, oral mucosa, and genitourinary areas will subsequently occur lasting for 2 to 4 weeks. Localized worsening of the lesions usually follows and progresses to extensive lesions formation throughout the body. When areas of lesions enlarge and spread resulting 30% or more of the body surface peel off, this condition is called 'Toxic Epidermal Necrolysis' (TEN). In such case, the patients become very ill and are susceptible to complications leading to a mortality rate of up to 30%.

Table 1. Drugs commonly cause SJS

Source: Hong Kong and Drug Education Resources Centre, The Society of Hospital Pharmacists of Hong Kong

More stories