HLA TYPING IN PATIENTS WITH OCULAR MANIFESTATIONS OF STEVENS-JOHNSON SYNDROME

Background: Stevens-Johnson syndrome (SJS) is an acute, self-limited, inflammatory disorder of the skin and mucous membranes. With ocular in volvement, SJS has been associated with the class I human leukocyte an tigen (HLA)-Bw44, This study examined HL4 class II associations in pat ients with SJS with ocular involvement to help identify possible molec ular genetic mechanisms underlying disease susceptibility/resistance. Methods: Twenty-three white patients with ocular complications seconda ry to SJS had HL4 class II typing performed using polymerase chain rea ction-based molecular techniques. Genotype frequency was compared with results obtained from 175 control subjects, Results: HLA-DQB10601 wa s present in four (17%) patients with SJS and in five (3%) control sub jects (P < 0.05; relative risk = 7.2). There was no association with H LA-DQB10301, which previously has been been strongly associated with recurrent erythema multiforme. None of the class II antigens tested ap peared to offer a protective effect against the development of disease . Conclusion: HLA-DQB10601 was found in a significantly disproportion ate number of white patients with SJS and ocular complications. The pr esence of this allele may confer an increased risk for the development of SJS with ocular complications and provides further evidence for an underlying immunogenetic susceptibility to the development of this di sease.