Steven-Johnson Syndrome in Different Skin Types


The topic of BAME (Black, Asian and Minority Ethnic) dermatology has been of increased interest this year. Social media activists and media organisations have united in their efforts to bridge the gap in dermatological literature and the guidelines, in order to make the speciality more inclusive and to increase the accuracy of diagnosis between varying skin types. Having said this, one must be acutely aware of the difficulties in changing guidelines and practice, predominantly because if we are to overcome these hurdles, we must first identify their origins. Hopefully, over time, as there is increased awareness, people can receive a more accurate diagnosis, and the quality of treatment provided will subsequently increase accordingly.

One such problem is the sheer difficulty in categorising people’s ethnicities and producing coherent guidelines, for there are a multitude of skin types and sub-categories that these could be split into, and this may initially cause confusion, misdiagnosis or even overdiagnosis. Another issue is that we are making a big assumption that all people within one category of skin will have the same dermatological presentation of a condition; this is not true, everyone’s skin is unique. This being said, it is important we are very aware of this, that textbooks are representative and varied in their images of skin, and that we know the differing presentations of any set skin condition so we can move forward as a speciality. A good place to start increasing awareness is in dermatological emergencies, as these may be life-threatening or severe and painful. 

One such condition is Stevens-Johnson Syndrome (SJS). Named after the two American paediatricians Albert Mason Stevens and Frank Chambliss Johnson, SJS was first reported in the American Journal of Diseases of Children in 1922, spotted in two children and described as an eruption with prolonged fever and inflamed buccal mucosa and severe purulent conjunctivitis. Although a rare condition, it is an important one to spot and to treat quickly and with urgency. The first case was reported in a child which persisted even after thirteen days of fever and although the picture is black and white, the child is quite clearly fair-skinned.1 Interestingly, however, the description of the rash has remained fairly similar since the very first case.

SJS is a serious condition and usually results from an allergic reaction to medication, begins with a fever and produces eruptions which cover 10% of the body area. If the area covered is greater than 30% then the condition is what is known as toxic epidermal necrolysis (TEN), which is even more severe and life-threatening. This highlights the importance of spotting SJS in all skin types so that it can be adequately treated. SJS is caused by numerous adverse drug reactions such as reactions to antibacterial sulfonamides, certain NSAIDs and certain antiepileptics, just to name a few. This being said, approximately one-quarter of cases originate from an unknown cause.2

One such case of SJS was in a teenager with BAME skin, built upon a background of severe pain, an oral temperature of 39.6ºC and an increased heart rate of 108 beats per minute, which is above the threshold of 100 beats per minute (tachycardia). The teenager had mucopurulent conjunctivitis with episcleritis and also severe mucosal inflammation of the lips. You can see how the condition is similar to that of the very first case of SJS, yet different in the appearance of the lesions themselves, with a more severe condition in the mucosal membranes. Whether or not the condition will be the same in all skin types with more melanin though is up for debate. The condition now is described as blistering and peeling of the skin but does this account for all skin types? Will all skin types blister and peel or is this just in the majority of children, and is this representative of all skin types? Currently, the literature and guidelines are written in such a way that makes SJS obvious in all skin types such that no one’s life is at risk, however, there need to be more case studies of strange presentations of SJS, particularly in patients with BAME skin, as with most other skin conditions, the more data the better and safer the quality of healthcare we can deliver. 

Another question is, was the treatment the BAME child received optimum for his condition and did it take people any longer to diagnose SJS in that young child than someone who was more fair-skinned? Actually, the child with BAME skin was diagnosed and treated in 2 days because his condition was so severe that he had to be taken to the emergency department!3 It’s hard to tell though, whether this is representative of all people with BAME skin, and whether they are disproportionately affected. 

Overall, it is important that we take multiple approaches in bridging the gap in dermatology between different skin types. One such method is by producing more case reports of interesting dermatological presentations in order not to miss these, because the heavier and more varied our data set, the better we will be at diagnosing and treating. Another is to do increased research into BAME skin presentations, to see whether one treatment plan is better in people with increased melanin than treatment plans in people with lower levels of melanin. Once we have increased the magnitude of our case reports, we can analyse large data sets and produce systematic reviews highlighting and discovering new patterns of the presentation of skin conditions within groups of people and this could be truly transformative in the field of dermatology. 

By; Armita Azar (3rd Year Medical Student)

References
1. https://jamanetwork.com/journals/jamapediatrics/article-abstract/1173827
2. https://rarediseases.org/rare-diseases/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis/
3. https://www.contemporarypediatrics.com/view/stevens-johnson-syndrome

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Last revised: 29 July 2020
Next review: 29 July 2023