Stevens-Johnson Syndrome (SJS)
Stevens-Johnson Syndrome (SJS) is a severe skin-mucosal reaction, predominantly caused by medications, characterized by blistering and widespread epidermal detachment. The systemic symptoms including multi-organ failure syndrome, and carries a high risk of mortality. Our company is well-equipped to address your drug and therapy development requirements in Stevens-Johnson Syndrome therapy.
Introduction to Stevens-Johnson Syndrome
Stevens-Johnson Syndrome is a rare but severe mucocutaneous condition often triggered by medications, with an incidence ranging between 1.2 to 12.35 new cases per million annually. This syndrome predominantly affects the skin and mucous membranes and can lead to significant morbidity and mortality (Frontiers). The global incidence of SJS, along with its more severe form, toxic epidermal necrolysis (TEN), is estimated to be up to 12 cases per million population each year.
Pathogenesis of Stevens-Johnson Syndrome
SJS often result from the cumulative effects of certain risk factors, including the molecular structure of the offending drug and specific human leukocyte antigen (HLA) alleles that predispose individuals to these reactions. The interaction between drugs and HLA alleles can lead to an immune response that targets and damages the skin and mucous membranes. Moreover, genetic variants such as those in the cytochrome P450 enzymes (CYP2C) have been linked to these conditions, affecting drug metabolism and exacerbating the toxicity that contributes to the development of SJS .
Fig. 1 "Swiss cheese" risk model of Stevens–Johnson syndrome and toxic epidermal necrolysis (SJS/TEN). (Dodiuk-Gad, R.P., et al., 2015)Diagnosis Development of Stevens-Johnson Syndrome
Recent studies have identified several new biomarkers for the diagnosis of Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. Researchers have pinpointed proteins such as galectin 7 and RIP3 as potential biomarkers that could be used for early detection of SJS/TEN. These findings are still in the early stages of research but offer promising new directions for the diagnosis of these severe skin conditions.
Therapy Development of Stevens-Johnson Syndrome
For example, corticosteroids are often used to reduce inflammation and suppress the immune response. Another class, anticonvulsants like cyclosporine, are employed to modulate immune system activity, potentially reducing the severity of reactions.
For SJS, therapies like mesenchymal stem cells (MSCs) are being researched for their ability to modulate immune responses and promote tissue healing. MSCs can differentiate into skin cells and help repair damaged tissues, offering a regenerative solution to the necrosis seen in SJS.
In the context of SJS, monoclonal antibodies such as infliximab may be used to target and neutralize TNF-alpha, a cytokine heavily involved in the inflammatory processes of SJS, potentially reducing the severity of the symptoms.
Gene therapy aims to correct or mitigate genes that might predispose individuals to severe reactions. Gene editing technologies like CRISPR/Cas9 could potentially be used to modify the HLA genes associated with drug hypersensitivity, thereby reducing the risk of developing SJS.
Our Services
Our company adopts a partnership-driven approach. We collaborate closely with clients to craft tailored, innovative Stevens-Johnson Syndrome therapy strategies and ensure robust support throughout the process.
Platforms of Stevens-Johnson Syndrome Therapy Development
Animal Models of Stevens-Johnson Syndrome
We have established expertise in developing and utilizing relevant animal models that closely mimic the disease characteristics and response to therapy. These models enable us to evaluate the safety and efficacy of potential therapies.
| Non-Genetically Engineering Models | ||
| We provide diverse model choices customized to meet specific research needs related to Stevens-Johnson Syndrome. These models allow researchers to simulate and study the complex biological processes associated with Stevens-Johnson Syndrome. | ||
| Induced Disease Models | ||
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| Xenograft Models | ||
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| Genetically Engineered Models | ||
| Our expertise in genetic engineering techniques, such as CRISPR/Cas9 technology, allows us to generate accurate and reliable models that recapitulate the genetic alterations observed in human Stevens-Johnson Syndrome. | ||
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| Optional Species | Mice, Rats, Non-human primates, Others | |
In addition to these models, our comprehensive services encompass other models that target specific signaling pathways and molecular targets.
If our services align with your goals, please contact us for more details.
References
- Dodiuk-Gad, R.P., et al., "Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: An Update." Am J Clin Dermatol, (2015). 16(6): p. 475-493.
- Frantz, R., et al., "Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis: A Review of Diagnosis and Management." Medicina (Kaunas), (2021). 57(9).
- Azukizawa, H., "Animal models of toxic epidermal necrolysis." J Dermatol, (2011). 38(3): p. 255-260.
All of our services and products are intended for preclinical research use only and cannot be used to diagnose, treat or manage patients.