What is Steven - Johnson syndrome (SJS , SSJ) ??

Steven-Johnson syndrome (SJS , SSJ) is a collection of clinical symptoms mukokutaneus eruption characterized by a triad vesikulobulosa disorders of the skin, mucous orifice of the eyes accompanied by severe general symptoms. The synonym include: syndrome de Friessinger-Rendu, erythema multiform major eksudativum, erythema poliform bulosa, syndrome-kutaneo MUKO-ocular, dermatostomatitis, etc..


SJS Etiology determined with any certainty is difficult, because the causes of various factors, although in general is often associated with immune response to the drug. Several factors cause SJS including: infections (viruses, fungi, bacteria, parasites), drugs (salicylates, sulfa, penicillin, etambutol, tegretol, tetracyclines, digitalis, contraceptives), food (chocolate), physical (cold air, sunlight, X-rays), etc. (polagen disease, malignancy, pregnancy). Pathogenesis SJS until now not yet clear, although often associated with type III hypersensitivity reactions (immune complex reaction) caused by a complex of soluble antigens or metabolites with IgM and IgG antibodies and hypersensitivity reactions slow (delayed-type hypersensitivity reactions, type IV) is a reaction mediated by specific T lymphocytes.

Diagnosis directed to the appropriate manifestation of the triad of skin disorders, mucosa, eye, and its relationship to factors that cause clinically there is a target-shaped lesions, or eye sliced beef, abnormalities in the mucosa, fever. Also supported by laboratory tests including peripheral blood examination, examination immunologic, breeding germs and the resistance of the blood test and the lesion, and examination of skin biopsy hystopatologik. Anemia can be found in cases with severe bleeding, leukocytes are usually normal or slightly elevated, there is increased oenophiles. IgG and IgM levels can be high, C3 and C4 are normal or slightly decreased and can be detected circulating immune complexes. Skin biopsy was planned if there is no classic lesions. Imunoflurosesensi director can help diagnose atypical cases.