Steven Jhonson Syndrome
STEVENS-JOHNSON SYNDROME: PATHOPHYSIOLOGY, ETIOLOGY, DIAGNOSIS AND MANAGEMENT.
Roberto Carmona
Florida International University
Abstract: Steven Johnson Syndrome is an inmune disease charactherized by a detachment of the epidermis from dermis. It could be fatal and the pathophysilogy involves a complex hypersensitivity reaction with the participation of T lymphocytes that induce keratinocyte’s apoptosis. The syndrome can be cause by drigs, infections and malignancies. The diagnosis is difficlut due to the abscense of specific manifestations and laboratory tests. There is a genetic predisposition in individuals with certain HLA types. The disease
Overview: Stevens-Jonhson Syndrome (SJS)is an immune disease. The disorder was described as a delay hypersensitivity reaction with epidermal necrosis and the participation of infections, drugs and genetic factors. The clinical expression varies from a slight form to a serious systemic process that may implicate life-threatening complications and death. In spite of the differences in the severity of the manifestations, the etiology, pathophysiology and genetic influence remain the same (Hazim, 200). The disorder was reported for the first time by Stevens and Chambers in 1922, after observing a couple of boys with fever, diffuse rash and sores in the mouth and ocular mucosa. It was confused with measles. At the beginning of the 90’s after several investigations, the difference between Erythema Multiforme Major SJS was proposed. Further research revealed there were dissimilarities on the cutaneous lesion 's pattern, whereas EMM referred to target raised edematous papules. SJS was characterized by blisters on top of an erythematous or purpuric base ( Mockenhaupt et al., 2011).
Pathophysiology and Etiology: The development of a hypersensitivity reaction type 4 has been involved in the pathophysiology of the disease. There are groups of patients with certain conditions that lead to
Roberto Carmona
Florida International University
Abstract: Steven Johnson Syndrome is an inmune disease charactherized by a detachment of the epidermis from dermis. It could be fatal and the pathophysilogy involves a complex hypersensitivity reaction with the participation of T lymphocytes that induce keratinocyte’s apoptosis. The syndrome can be cause by drigs, infections and malignancies. The diagnosis is difficlut due to the abscense of specific manifestations and laboratory tests. There is a genetic predisposition in individuals with certain HLA types. The disease
Overview: Stevens-Jonhson Syndrome (SJS)is an immune disease. The disorder was described as a delay hypersensitivity reaction with epidermal necrosis and the participation of infections, drugs and genetic factors. The clinical expression varies from a slight form to a serious systemic process that may implicate life-threatening complications and death. In spite of the differences in the severity of the manifestations, the etiology, pathophysiology and genetic influence remain the same (Hazim, 200). The disorder was reported for the first time by Stevens and Chambers in 1922, after observing a couple of boys with fever, diffuse rash and sores in the mouth and ocular mucosa. It was confused with measles. At the beginning of the 90’s after several investigations, the difference between Erythema Multiforme Major SJS was proposed. Further research revealed there were dissimilarities on the cutaneous lesion 's pattern, whereas EMM referred to target raised edematous papules. SJS was characterized by blisters on top of an erythematous or purpuric base ( Mockenhaupt et al., 2011).
Pathophysiology and Etiology: The development of a hypersensitivity reaction type 4 has been involved in the pathophysiology of the disease. There are groups of patients with certain conditions that lead to
References: Hazim, R., Ibrahim, O., & Hazim, M. (2008). Stevens-Johnson syndrome: pathogenesis, diagnosis, and management. Annals of Medicine, 40(2), 129-138 Ko, T., Chung, W., Wei, C., Shih, H., Chen, J. et al. (2011, December). Shared and restricted T- cell receptor use is crucial for carbamazepine-induced Stevens-Johnson syndrome. The Journal of allergy and clinical immunology 128. 6, 128(6), 1266-1276. Retrieved July 3, 2012 from http://www.ncbi.nlm.nih.gov/pubmed/21924464?dopt=AbstractPlus Mockenhaupt, M. M. (2011). The current understanding of stevens-johnson syndrome and toxic epidermal necrolysis. Expert Review of Clinical Immunology, 7(6), 803-13; quiz 814-5 Paiz, J. M., Angeli, E., Wagner, J., Lawrick, E., Moore, K., Anderson, M., & Soderlund, L. (2012, May 30) Phillips, E. J. E. J., & Mallal, S. A. S. A. (2011). HLA-B*1502 screening and toxic effects of carbamazepine. United States: Retrieved from http://ezproxy.fiu.edu/login?url=http://search.proquest.com/docview/884423149?accountid=10901