REVIEW ARTICLE ON STEVENS JOHNSON SYNDROME

Abstract

Stevens-Johnson Syndrome (SJS) is rare, cutaneous, severe, drug-induced hypersensitivity reactions marked by widespread inflammation of the epidermis, ending in necrosis and the eventual sloughing of tissue.It is the syndrome associated with a rare, serious disorder of the skin, mucous membranes, genitals and eyes  usually a reaction to a medication or an infection. There are drugs that have been linked to Stevens - Johnson syndrome, and these include some NSAIDS (non-steroid anti-inflammatory drugs), Allopurinol, Phenytoin, Carbamazepine, barbiturates, anticonvulsants, and sulfa antibiotics. The condition can sometimes attributed to a infection such as Mycoplasma, cytomegalovirus and in some cases there is no known cause for the onset of this Syndrome. Histopathological hallmark of this disease is wide­spread epidermal necrosis due to death by apoptosis of keratinocytes. The pathogenesis of SJS has not been completely solved, but specific genetic predispositions, which vary among ethnic groups and differ between certain causing drugs, were identified. A positive Nikolsky sign is helpful in the diagnosis of SJS. Drug provocation tests are contraindicated, since a subsequent exposure to the agent could trigger a new se­vere episode of SJS.Complete blood count (CBC) may show unspecific leukocytosis or even indicate superimposed secondary bacterial infection. Cultures of blood, urine and skin can reveal the agent of the underlying suspected infection. Skin biopsy is an additional final examination. Complications include dehydration, sepsis, pneumonia, and multiple organ failure. Genetic research has identified several combinations of alleles and medications which when combined increase the risk of illness. Understanding pathophysiology is key for treating and limiting the destructive mechanism for disease. The success of  treatment depends on early recognition of the condition,  removal of the causative medications and intensive supportive care in a well-equipped hospital.

Keywords: Stevens Johnson syndrome, acetylators, Nikolskysign, Sepsis, Keratinocytes