Read more about this disease, some with Classification – Types – Signs and symptoms – Genetics – Pathophysiology – Diagnosis – Screening – Prevention – Treatment and management – Cures and much more, some including pictures and video when available.
Stevens-Johnson syndrome (SJS) is a life-threatening condition affecting the skin in which cell death causes the epidermis to separate from the dermis. The syndrome is thought to be a hypersensitivity complex affecting the skin and the mucous membranes. Although the majority of cases are idiopathic, the main class of known causes is medications, followed by infections and (rarely) cancers.
There is agreement in the medical literature that Stevens-Johnson syndrome can be considered a milder form of toxic epidermal necrolysis (TEN). However, there is debate whether it falls on a spectrum of disease that includes erythema multiforme and erythema multiforme major (also known as erythema multiforme majus).[1][2][3][4][5]
SJS is a rare condition, with a reported incidence of around one case per million people per year. In the United States, there are about 300 new diagnoses per year.[citation needed]
SJS can be caused by infections (usually following viral infections such as herpes simplex virus, influenza, mumps, cat-scratch fever, histoplasmosis, Epstein-Barr virus, or similar), adverse effects of drugs (allopurinol, diclofenac, fluconazole,[6] valdecoxib, sitagliptin, penicillins, barbiturates, sulfonamides, phenytoin, azithromycin, modafinil[7], lamotrigine, nevirapine, ibuprofen[8], ethosuximide, carbamazepine)[9][10], malignancy (carcinomas and lymphomas), or idiopathic factors (up to 50% of the time). SJS has also been consistently reported as an uncommon side effect of herbal supplements containing ginseng. SJS may also be caused by cocaine usage.[11]
Although Stevens-Johnson Syndrome can be caused by viral infections, malignancies or severe allergic reactions to medication, the leading cause appears to be the use of antibiotics and sulfa drugs. Medications that have traditionally been known to lead to Stevens-Johnson Syndrome, Erythema Multiforme, Lyell’s Syndrome, and Toxic Epidermal Necrolysis include sulfonamides (antibiotics), penicillins (antibiotics), barbiturates (sedatives), lamotrigine (anticonvulsant), and phenytoin – Dilantin (anticonvulsant). Combining lamotrigine with sodium valproate increases the risk of Stevens-Johnson Syndrome occurring.
In East Asians the carbamazepine and phenytoin induced syndrome is strongly associated with HLA-B75 (B*1502 allele of HLA-B).[12][13][14] A study in Europe suggested that the gene-marker is only relevant for East Asians.[15][16] Based on the Asian findings similar studies in Europe were performed, sixty-one percent of allopurinol-induced SJS/TEN carried the HLA-B58 (B*5801 allele – Phenotype frequency in Europeans is typically 3%), indicating that while the risk alleles differ between ethnicities, the HLA-B locus is strongly associated or closely linked to a strongly associated gene.[17]
[tubepress mode=’tag’, tagValue=’Stevens-Johnson syndrome’]