Adverse Drug Reactions and Stevens-Johnson Syndrome Guidelines

Introduction to Stevens-Johnson Syndrome

Stevens-Johnson Syndrome (SJS) is primarily caused by medications, with drugs responsible for approximately 90% of cases in adults, while in children both medications and infections are important triggers with up to 50% of pediatric cases attributed to infectious causes 1
Both infections and drugs are important triggers for SJS, with up to 50% of cases may be related to infections, according to the British Journal of Dermatology 1
Anticonvulsants and antibiotics are most commonly implicated medications in SJS, as noted by the British Journal of Dermatology 1

Anticonvulsants such as Carbamazepine, lamotrigine, phenytoin, and phenobarbital are associated with SJS, with a strong association reported by the British Journal of Dermatology 1, 2
Antibiotics like Sulfamethoxazole and other sulfonamides are linked to SJS, according to the British Journal of Dermatology 1
Other high-risk medications include Allopurinol 2, Nevirapine and other antiretrovirals 2, Oxicam-type NSAIDs 1, and Sulfasalazine 1

The following medications are associated with a high risk of SJS/TEN:

Medication Class	Examples
Antibiotics	Sulfamethoxazole, aminopenicillins, cephalosporins, quinolones
Anticonvulsants	Carbamazepine, lamotrigine, phenytoin, phenobarbital
Other	Allopurinol, nevirapine, oxicam-type NSAIDs, sulfasalazine

Most reactions occur within 5-28 days after starting the medication, as stated by the British Journal of Dermatology 2
A shorter latency period may occur in patients with previous exposure to the same drug, according to the British Journal of Dermatology 2

Mycoplasma pneumoniae is responsible for up to 50% of infection-related SJS cases in children, as noted by the British Journal of Dermatology 1
Herpes Simplex Virus (HSV) is also a trigger for SJS in the pediatric population, according to the British Journal of Dermatology 1
Other respiratory infections can also cause SJS in children, as stated by the British Journal of Dermatology 1
A specific variant of SJS/TEN secondary to respiratory infection has been described, involving predominantly mucous membranes with limited cutaneous lesions, termed "Mycoplasma pneumoniae-associated mucositis" or "Mycoplasma pneumoniae-induced rash and mucositis" 1

The ALDEN algorithm has been developed to help define drug causality in SJS/TEN, considering parameters such as timeline of drug exposure, previous exposure, drug notoriety, and presence of alternative causes, as recommended by the British Journal of Dermatology 1
Early recognition, immediate withdrawal of causative agents, and prompt referral to specialized care are essential for improving outcomes in SJS/TEN, according to the British Journal of Dermatology 1
The SCORTEN score should be calculated within the first 24 hours of admission to predict mortality, as recommended by the British Journal of Dermatology 2
Immediate withdrawal of the suspected medication is crucial as it decreases the risk of death, according to the British Journal of Dermatology 1
Document all medications taken by the patient in the 2 months prior to symptom onset, establish a timeline for each medication, and immediately discontinue any suspected medication, as recommended by the British Journal of Dermatology 2, 1
The British Journal of Dermatology recommends promptly referring the patient to a specialized unit, preferably a burn unit or ICU with experience in managing SJS/TEN 2

Patients with malignancy or stem cell transplantation have a worse prognosis with drug-induced SJS/TEN, as noted by the British Journal of Dermatology 1
Patients with specific HLA types have increased risk with certain medications, although the exact HLA types are not specified in this context due to the instruction to ignore certain references 1
HLA-B*1502 has a strong association with carbamazepine-induced SJS/TEN in patients of Southeast Asian ancestry, as reported by the British Journal of Dermatology 1
The British Journal of Dermatology recommends HLA typing in patients of Southeast Asian descent before initiating treatment with carbamazepine due to the strong association between the HLA-B*1502 genetic marker and carbamazepine-induced SJS/TEN 1
Nevirapine has a very high risk with a relative risk >22, as reported by the MMWR Recommendations and Reports 3
Efavirenz is associated with rash that can progress to SJS, as noted by the MMWR Recommendations and Reports 3
In patients with a history of SJS/TEN, it is recommended to avoid not only the causative medication but also those with similar chemical structures, as noted by the Journal of Allergy and Clinical Immunology 4