Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe hypersensitivity reactions of the skin. Most are drugs v. a. Sulfonamides, anti-epileptic drugs and antibiotics that trigger. The macules spreading rapidly and merge, so that there is epidermal blisters, necrosis and exfoliation of the epidermis. The diagnosis is obvious mostly by the appearance of the initial lesions and the clinical syndrome. The treatment is supportive care; Cyclosporine, plasma exchange or IVIG and an early pulse corticosteroid therapy are applied. The mortality can range in adults is up to 7.5% in children and 20 to 25%, but is lower in early treatment.
SJS and TEN are apart from their distribution clinically similar. After a generally accepted definition of the lesions affecting <10% of the body surface and TEN> 30% of the body surface at SJS. An involvement of 15-30% of the body surface is considered overlap SJS / TEN.
Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe hypersensitivity reactions of the skin. Most are drugs v. a. Sulfonamides, anti-epileptic drugs and antibiotics that trigger. The macules spreading rapidly and merge, so that there is epidermal blisters, necrosis and exfoliation of the epidermis. The diagnosis is obvious mostly by the appearance of the initial lesions and the clinical syndrome. The treatment is supportive care; Cyclosporine, plasma exchange or IVIG and an early pulse corticosteroid therapy are applied. The mortality can range in adults is up to 7.5% in children and 20 to 25%, but is lower in early treatment. SJS and TEN are apart from their distribution clinically similar. After a generally accepted definition of the lesions affecting <10% of the body surface and TEN> 30% of the body surface at SJS. An involvement of 15-30% of the body surface is considered overlap SJS / TEN. The diseases occur in 1-5 of a million people. Incidence and / or severity of both diseases can be higher with other chronic rheumatic diseases in recipients of bone marrow transplants in HIV patients with Pneumocystis jirovecii infection in SLE patients and in patients. Etiology drugs are responsible for 50% of SJS cases and up to 95% of TEN cases. The most commonly-causing drugs include sulfonamides (z. B. cotrimoxazole, sulfasalazine) Other antibiotics (eg. As Aminopenicillins [usually ampicillin or amoxicillin], fluoroquinolones, cephalosporins) antiepileptic drugs (eg., Phenytoin, carbamazepine, phenobarbital, valproate, lamotrigine) Other individual drugs (eg. as piroxicam, allopurinol, chlormezanone) cases that are not caused by medication are attributed to infection (usually with Mycoplasma pneumoniae) immunizations graft-versus-host disease in rare cases, no cause be determined. Pathophysiology The exact pathophysiological mechanism is unknown. One theory suggests that an altered drug metabolism triggers (z. B. an inability to clarify reactive metabolites) in some patients a T-cell mediated cytotoxic response to the drug antigens in keratinocytes. CD8 + T cells have been identified as important mediators of blistering. Recent results suggest that granulysin, which was released by cytotoxic T cells and natural killer cells, may play a role in the death of keratinocytes; the Granulysinkonzentration in the blister fluid correlated with the severity of the disease. Another theory is that interaction between Fas (a cell surface receptor, apoptosis causes) and its ligand, particularly a soluble form that is released from mononuclear cells of the Fas ligand, result in cell death and the formation of blisters. A genetic predisposition to SJS / TEN has been proposed. Symptoms and complaints within 1-3 weeks after initiation of therapy with the drug causing develop the patient prodrome with malaise, fever, headache, cough and keratoconjunctivitis. Then often kokardenförmige macules suddenly occur mostly on the face, neck and upper body. At the same time these macules also appear elsewhere on the body to merge into large, flat bullae and peel off over 1-3 days. Together with the epithelium also nails and eyebrows may be lost. The palms and soles may be involved. In some cases a diffuse redness is the first skin abnormality of TEN. Stevens-Johnson syndrome DR P. arazzi / SCIENCE PHOTO LIBRARY var model = {thumbnailUrl: ‘/-/media/manual/professional/images/m2600088-stevens-johnson-syndrome-science-photo-library-high_de.jpg?la = en & thn = 0 & mw = 350 ‘, imageUrl:’ /-/media/manual/professional/images/m2600088-stevens-johnson-syndrome-science-photo-library-high_de.jpg?la=de&thn=0 ‘, title:’ Stevens-Johnson syndrome ‘, description:’ u003Ca id = “v37894824 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “”para “” u003e u003cp u003eDiese Figure shows a reddened skin rash and blisters on the skin and on the mucous membranes of the eyes and mouth in this patient with SJS u003c / p u003e u003c / div u003e ‘credits’. DR P. arazzi / SCIENCE PHOTO LIBRARY’