Adverse Reactions & Pharmacovigilance

Stevens–Johnson Syndrome SJS

TRStevens–Johnson Sendromu

A rare, severe, usually drug-induced mucocutaneous reaction with widespread keratinocyte death, blistering and mucosal erosions, in which epidermal detachment involves less than 10% of body surface area.

In depth

SJS and toxic epidermal necrolysis (TEN) form one disease spectrum graded by the extent of detachment: SJS below 10%, SJS/TEN overlap 10–30% and TEN above 30% of body surface area. The mechanism is a drug-specific cytotoxic T-cell response that triggers keratinocyte apoptosis, mediated largely by granulysin and Fas ligand. A febrile, flu-like prodrome typically precedes the rash, which usually appears within the first eight weeks of therapy.

High-risk drugs include allopurinol, carbamazepine, lamotrigine, phenytoin, phenobarbital, sulfonamide antibiotics, nevirapine and oxicam NSAIDs. HLA associations such as HLA-B*15:02 with carbamazepine (strongest in Southeast and South Asian ancestry) and HLA-B*58:01 with allopurinol allow pre-emptive screening. The SCORTEN score estimates mortality, which is substantially higher in TEN.

Clinical pearl

Early withdrawal of the culprit drug improves survival; the patient must never be re-exposed, and structurally related drugs (e.g., other aromatic anticonvulsants) should be avoided.

Example drugs

Carbamazepine Allopurinol Lamotrigine Phenytoin Sulfamethoxazole Nevirapine
TRIn Turkish

Stevens–Johnson Sendromu

Yaygın keratinosit ölümü, büller ve mukozal erozyonlarla seyreden, epidermal ayrışmanın vücut yüzey alanının %10'undan azını tuttuğu, seyrek görülen, ağır ve çoğunlukla ilaca bağlı mukokutanöz reaksiyondur.

SJS ve toksik epidermal nekroliz (TEN), ayrışan alanın genişliğine göre derecelendirilen tek bir hastalık yelpazesi oluşturur: vücut yüzey alanının %10'undan azı SJS, %10–30'u SJS/TEN örtüşmesi, %30'undan fazlası TEN sayılır. Mekanizma, büyük ölçüde granülizin ve Fas ligandı aracılığıyla keratinosit apoptozunu tetikleyen ilaca özgü sitotoksik T hücre yanıtıdır. Genellikle ateşli, grip benzeri bir prodromu izleyen döküntü çoğunlukla tedavinin ilk sekiz haftasında ortaya çıkar.

Yüksek riskli ilaçlar arasında allopurinol, karbamazepin, lamotrijin, fenitoin, fenobarbital, sülfonamid antibiyotikler, nevirapin ve oksikam grubu NSAİİ'ler yer alır. Karbamazepinle HLA-B*15:02 (özellikle Güneydoğu ve Güney Asya kökenlilerde) ve allopurinolle HLA-B*58:01 gibi HLA ilişkileri önleyici taramaya olanak tanır. SCORTEN skoru mortaliteyi öngörür; mortalite TEN'de belirgin biçimde yüksektir.

Sorumlu ilacın erken kesilmesi sağkalımı iyileştirir; hasta bu ilaca asla yeniden maruz bırakılmamalı, yapısal olarak benzer ilaçlardan (ör. diğer aromatik antikonvülzanlar) da kaçınılmalıdır.

Updated 24.09.2026 0 views Educational reference — verify against current sources before clinical use.

Read next

HLA-B*15:02HLA-B*15:02

A human leukocyte antigen allele strongly associated with carbamazepine-induced Stevens–Johnson syndrome and toxic epidermal necrolysis, most prevalent in Southeast and South Asian ancestry.

 Drug Hypersensitivityİlaç Aşırı Duyarlılığı

An adverse drug reaction that clinically resembles allergy; when a specific immunological mechanism (IgE or T cells) is demonstrated it is termed drug allergy, otherwise non-allergic hypersensitivity.

 DRESS SyndromeDRESS Sendromu

A severe, delayed, T-cell-mediated drug hypersensitivity syndrome with fever, extensive rash, lymphadenopathy, haematological abnormalities such as eosinophilia, and internal organ involvement.

 Cross-ReactivityÇapraz Reaktivite

The occurrence of a hypersensitivity reaction to a drug in a patient sensitised to a different drug, because both share a structural determinant or trigger the same reaction pathway.

 Type B Adverse ReactionTip B Advers Reaksiyon

An adverse drug reaction that is not expected from the drug's known pharmacology, is largely independent of dose, and is uncommon but frequently serious.

 Serious Adverse EventCiddi Advers Olay

An adverse event that results in death, is life-threatening, requires or prolongs hospitalisation, causes persistent or significant disability, is a congenital anomaly, or is otherwise medically important.