Familial Occurrence of Stevens Johnson Syndrome

Fischer, Philip R.

doi:10.1001/archpedi.1983.02140350086023

Cited by 14 publications

(11 citation statements)

References 11 publications

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“…These drugs must never be re-administered. Counselling of first-degree relatives is advised in the light of evidence from familial cases to suggest that they may be at increased risk of developing adverse reactions to the same drugs 42. The wearing of a medical alert bracelet, detailing the patient’s drug sensitivity, is an advisable measure.…”

Section: Prognosismentioning

confidence: 99%

Management of toxic epidermal necrolysis and related syndromes

Mukasa

Craven

2008

Postgraduate Medical Journal

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Toxic epidermal necrolysis and Stevens-Johnson syndrome are rare and life-threatening diseases that often configure as medical emergencies. The majority of cases are drug reactions. The clinical picture is one of widespread epidermal necrosis and mucosal erosions. Treatment is largely supportive and must be provided in an appropriate environment. The role of steroids and other potential disease-modifying therapies has yet to be fully established by controlled studies. The significant mortality associated with these conditions dictates that an understanding of these conditions is essential for all doctors.

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Section: Prognosismentioning

confidence: 99%

Management of toxic epidermal necrolysis and related syndromes

Mukasa

Craven

2008

Postgraduate Medical Journal

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“…Prior studies on the genetic susceptibility Familial occurrences of SJS, including cases occurring in identical twins, have been reported [45][46][47]. Carbamazepine hypersensitivity has also been observed in monozygotic twins [48].…”

Section: Microsomal Epoxide Hydrolasementioning

confidence: 99%

HLA-B Genotyping to Detect Carbamazepine-Induced Stevens-Johnson Syndrome: Implications for Personalizing Medicine

Hung

Chung

Chen

2005

Personalized Medicine

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Preventing severe adverse drug reactions by identifying people at risk with a simple genetic test is the goal of many pharmacogenomic studies. Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are related, life-threatening cutaneous adverse reactions, most often caused by medication. The overall incidence and the commonly offending drugs vary among different ethnic populations. Susceptibility to such idiosyncratic reactions is thought to be genetically determined and immune mediated. Finding a strong genetic association between a particular human leukocyte antigen (HLA)-B allele and the reaction to a specific drug provides evidence that the pathogenesis of the severe cutaneous adverse drug reactions involves major histocompatibility complex-restricted presentation of a drug or its metabolites for T-cell activation. In the case of carbamazepine-induced SJS/TEN, the tight association of the HLA-B*1502 allele (sensitivity 100%, specificity 97% and odds ratio 2504) provides a plausible basis for further development of such a test to identify individuals at risk of developing this life-threatening condition.

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“…In an era when HLA typing was performed by serology, weak two-digit associations were described for allopurinol (B58) and sulfa antimicrobial hypersensitivity. 45–47 The strong association between HLA-B*57:01 and the abacavir hypersensitivity syndrome was identified in 2002 and marked the advent of a new era of discovery made possible by high resolution HLA typing in patients with specifically phenotyped drug hypersensitivity syndromes. 48,49 This discovery was followed by the reported associations between carbamazepine SJS/TEN and HLA-B*15:02 and allopurinol DRESS/SJS/TEN and HLA-B*58:01 48,50–52 (Table 1).…”

Section: Genetic Associations Of Im-adrsmentioning

confidence: 99%