Adult-onset immunodeficiency due to anti-interferon-γ autoantibody is an emerging acquired immunodeficiency with frequent skin manifestations. A retrospective chart review was conducted and identified 41 patients with the syndrome. Skin involvement was detected in 33 (80%) patients, 15 (45%) with infective skin diseases and 27 (82%) with reactive skin disorders. Reactive lesions were mostly neutrophilic dermatoses, e.g. Sweet syndrome. Of note, the presence of neutrophilic dermatoses was highly associated with infections of other sites. An adjusted odds ratio for the existence of infections in patients with neutrophilic dermatoses was 14.79 (95% CI: 5.13, 42.70; p < 0.001). Moreover, neutrophilic dermatoses were significantly correlated with opportunistic infections observed in those with defects in cell-mediated immunity including non-tuberculous mycobacterium and disseminated fungal infection. The odds ratio for opportunistic infections in the presence of neutrophilic dermatoses was 12.35 (95% CI: 5.00, 30.55; p <0.001). Thus, the presence of neutrophilic dermatoses in patients with the syndrome can signal opportunistic infections that warrant physician attention.

No abstract

No abstract

RATIONALE: Interferon gamma (IFN-g) enzyme-linked immunospot (ELISpot) assay has been introduced for drug hypersensitivity diagnosis, however, the cut-off value is not yet optimized. The aim is to determine the clinical cut-off value of IFN-g ELISpot assay in patients with a history of drug hypersensitivity based on drug challenge test and/or skin test results. METHODS: The frequencies of IFN-g releasing cells after stimulating peripheral blood mononuclear cells (PBMC) with 72 suspected culprit drug(s) in 54 patients with a history of drug hypersensitivity were comparatively analyzed with the results of drug challenge test and/or skin test. The optimal cut-off value was calculated based on the receiver operating characteristic (ROC) curve results. RESULTS: About 40.7% (22/54) of patients in this study have a history of severe cutaneous adverse reactions (SCARs) including 8 drug reaction with eosinophilia and systemic symptoms, 5 Stevens-Johnson syndrome/ toxic epidermal necrolysis, and 9 acute generalized exanthematous pustulosis. The average frequencies of drug-induced IFN-g releasing cells in confirmed drug hypersensitivity group were significantly higher than those in drug tolerant group (70.2623.4 and 13.7610.8 cells/10 6 PBMC), respectively; p value50.04). According to ROC curve analysis, the cut-off value of 22 cells/10 6 PBMC yielded 65.2% sensitivity, 95.9% specificity, and excellent positive likelihood ratio (15.9). The sensitivity and specificity increased to 77.8% and 100.0%, respectively, in drug-induced SCAR subjects. CONCLUSIONS: Our study confirms that IFN-g ELISpot assay has good clinical diagnostic values to identify the culprit drug, especially in SCARs. The suspected culprit drugs with positive ELISpot result should be avoided from further use due to high specificity of the test.79 Hypersensitivity reactions to antituberculosis drugs confirmed by interferon gamma enzymelinked Immunospot assay