title: NF-κB Essential Modulator Deficiency Leading to Disseminated Cutaneous Atypical Mycobacteria.
abstract: NF-κB essential modulator (NEMO) is a kinase integral to the macrophage TNF-α pathway, which leads to the intracellular destruction of Mycobacteria species. Defects in the NEMO pathway result in spectrum of diseases, including but not limited to ectodermal dysplasia, Mendelian susceptibility to mycobacterial diseases, and incontinentia pigmenti. In addition, paucity of NEMO can lead to the inability to mount a proper immune response against opportunistic pyogenic and mycobacterial infections, leading to dissemination to various organ systems. This manuscript will discuss the numerous clinical manifestations of NEMO deficiency, the differential diagnosis of atypical mycobacterial infections in immunocompetent adults, and feature a case report of rare isolated susceptibility to disseminated atypical mycobacteria due to a mutation in the first exon of the NEMO gene.
authors.affiliation: Morsani College of Medicine, University of South Florida, Tampa, Florida.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Braue
authors.affiliation: Division of Infectious Diseases, Moffitt Cancer Center, Tampa, Florida.
authors.forename: Vagishwari
authors.initials: V
authors.lastname: Murugesan
authors.affiliation: Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland.
authors.forename: Steven
authors.initials: S
authors.lastname: Holland
authors.affiliation: Department of Dermatology and Cutaneous Surgery, Morsani College of Medicine, University of South Florida, Tampa, Florida.
authors.forename: Nishit
authors.initials: N
authors.lastname: Patel
authors.affiliation: Division of Infectious Disease and International Medicine, Morsani College of Medicine, University of South Florida, Tampa, Florida.
authors.forename: Eknath
authors.initials: E
authors.lastname: Naik
authors.affiliation: Department of Pediatrics, Division of Allergy, Immunology, and Rheumatology, Morsani College of Medicine, University of South Florida, Tampa, Florida.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Leiding
authors.affiliation: Division of Infectious Diseases, Moffitt Cancer Center, Tampa Florida.
authors.forename: Abraham Tareq
authors.initials: AT
authors.lastname: Yacoub
authors.affiliation: Department of Dermatology, Morsani College of Medicine, University of South Florida, Tampa, Florida.
authors.forename: Carlos N
authors.initials: CN
authors.lastname: Prieto-Granada
authors.affiliation: Chief, Division of Infectious Diseases, Moffitt Cancer Centre, Tampa.
authors.forename: John Norman
authors.initials: JN
authors.lastname: Greene