Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content +++ Key Features ++ Recurrent serious infections "Cold" abscesses without pus formation Poor wound healing Gingival or periodontal disease (or both) +++ Clinical Findings ++ Patients present with variably severe phenotypes, including Recurrent serious infections Lack of pus formation Poor wound healing Gingival and periodontal disease The hallmark is little inflammation and absent neutrophils on histopathologic evaluation of infected sites (ie, "cold" abscesses), especially when concurrent with neutrophilia, and expression of poor adherence to vessel walls The most severe phenotype manifests with infections in the neonatal period, including delayed separation of the umbilical cord with associated omphalitis +++ Diagnosis ++ Striking neutrophilia demonstrated on laboratory evaluation Diagnosis is confirmed by flow cytometry analysis for CD18 (type I) or CD15s (type II) +++ Treatment ++ Aggressive antibiotic therapy Fucose supplementation in type II has been reported with some success Your MyAccess profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Username Error: Please enter User Name Password Error: Please enter Password Forgot Username? Forgot Password? Sign in via OpenAthens Sign in via Shibboleth